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Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management

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Abstract

Autoimmune subepidermal blistering diseases of the skin and mucosae constitute a large group of sometimes devastating diseases, encompassing bullous pemphigoid, gestational pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. Their clinical presentation is polymorphic. These autoimmune blistering diseases are associated with autoantibodies that target distinct components of the basement membrane zone of stratified epithelia. These autoantigens represent structural proteins important for maintenance of dermo-epidermal integrity. Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering disease of the skin and mucosae. Although the disease typically presents with a generalized blistering eruption associated with itch, atypical variants with either localized bullous lesions or “non-bullous” presentations are observed in approximately 20% of patients. A peculiar form of BP typically associated with pregnancy is pemphigoid gestationis. In anti-p200 pemphigoid, patients present with tense blisters on erythematosus or normal skin resembling BP, with a predilection for acral surfaces. These patients have antibodies targeting the 200-kDa basement membrane protein. Epidermolysis bullosa is a rare autoimmune blistering disease associated with autoantibodies against type VII collagen that can have several phenotypes including a classical form mimicking dystrophic epidermolysis bullosa, an inflammatory presentation mimicking BP, or mucous membrane pemphigoid-like lesions. Mucous membrane pemphigoid (MMP) is the term agreed upon by international consensus for an autoimmune blistering disorder, which affects one or more mucous membrane and may involve the skin. The condition involves a number of different autoantigens in the basement membrane zone. It may result in severe complications from scarring, such as blindness and strictures. Diagnosis of these diseases relies on direct immunofluorescence microscopy studies and immunoserological assays. Management of affected patients is often challenging. We will here review the clinical and immunopathological features as well as the pathophysiology of this group of organ-specific autoimmune diseases. Finally, we will discuss the diagnostic approach and the principles of management in clinical practice.

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Abbreviations

AIBD:

autoimmune blistering disease

BMZ:

basement membrane zone

BP:

bullous pemphigoid

CNS:

central nervous system

DEJ:

dermo-epidermal junction

DIF:

direct immunofluorescence

EBA:

epidermolysis bullosa acquisita

IEM:

immunoelectron microscopy

MMP:

mucous membrane pemphigoid

PUVA:

Psoralen and ultraviolet A

PG:

pemphigoid gestationis

OCP:

ocular cicatricial pemphigoid

FOAM:

fluorescent overlay antigenic mapping

References

  1. Schmidt E, Zillikens D (2013) Pemphigoid diseases. Lancet 381:320–332. doi:10.1016/s0140-6736(12)61140-4

    Article  PubMed  Google Scholar 

  2. Serwin AB, Musialkowska E, Piascik M (2014) Incidence and mortality of bullous pemphigoid in north-east Poland (Podlaskie Province), 1999-2012: a retrospective bicentric cohort study. Int J Dermatol 53:e432–e437. doi:10.1111/ijd.12492

    Article  PubMed  Google Scholar 

  3. Forsti AK, Jokelainen J, Timonen M, Tasanen K (2014) Increasing incidence of bullous pemphigoid in Northern Finland: a retrospective database study in Oulu University Hospital. Br J Dermatol 171:1223–1226. doi:10.1111/bjd.13189

    Article  PubMed  Google Scholar 

  4. Brick KE, Weaver CH, Lohse CM, Pittelkow MR, Lehman JS, Camilleri MJ, Al-Hashimi M, Wieland CN (2014) Incidence of bullous pemphigoid and mortality of patients with bullous pemphigoid in Olmsted County, Minnesota, 1960 through 2009. J Am Acad Dermatol 71:92–99. doi:10.1016/j.jaad.2014.02.030

    Article  PubMed Central  PubMed  Google Scholar 

  5. Marazza G, Pham HC, Scharer L, Pedrazzetti PP, Hunziker T, Trueb RM, Hohl D, Itin P, Lautenschlager S, Naldi L, Borradori L (2009) Incidence of bullous pemphigoid and pemphigus in Switzerland: a 2-year prospective study. Br J Dermatol 161:861–868. doi:10.1111/j.1365-2133.2009.09300.x

    Article  CAS  PubMed  Google Scholar 

  6. Bertram F, Brocker EB, Zillikens D, Schmidt E (2009) Prospective analysis of the incidence of autoimmune bullous disorders in Lower Franconia, Germany. J Dtsch Dermatol Ges 7:434–440. doi:10.1111/j.1610-0387.2008.06976.x

    PubMed  Google Scholar 

  7. Langan SM, Smeeth L, Hubbard R, Fleming KM, Smith CJ, West J (2008) Bullous pemphigoid and pemphigus vulgaris—incidence and mortality in the UK: population based cohort study. BMJ 337:a180. doi:10.1136/bmj.a180

    Article  CAS  PubMed  Google Scholar 

  8. Gudi VS, White MI, Cruickshank N, Herriot R, Edwards SL, Nimmo F, Ormerod AD (2005) Annual incidence and mortality of bullous pemphigoid in the Grampian region of North-east Scotland. Br J Dermatol 153:424–427. doi:10.1111/j.1365-2133.2005.06662.x

    Article  CAS  PubMed  Google Scholar 

  9. Joly P, Baricault S, Sparsa A, Bernard P, Bedane C, Duvert-Lehembre S, Courville P, Bravard P, Remond B, Doffoel-Hantz V, Benichou J (2012) Incidence and mortality of bullous pemphigoid in France. J Invest Dermatol 132:1998–2004. doi:10.1038/jid.2012.35

    Article  CAS  PubMed  Google Scholar 

  10. Jung M, Kippes W, Messer G, Zillikens D, Rzany B (1999) Increased risk of bullous pemphigoid in male and very old patients: a population-based study on incidence. J Am Acad Dermatol 41:266–268

    Article  CAS  PubMed  Google Scholar 

  11. Hubner F, Recke A, Zillikens D, Linder R, Schmidt E (2016) Prevalence and age distribution of pemphigus and pemphigoid diseases in Germany. J Invest Dermatol 136:2495–2498. doi:10.1016/j.jid.2016.07.013

    Article  PubMed  CAS  Google Scholar 

  12. Lee JH, Kim SC (2014) Mortality of patients with bullous pemphigoid in Korea. J Am Acad Dermatol 71:676–683. doi:10.1016/j.jaad.2014.05.006

    Article  PubMed  Google Scholar 

  13. Cai SC, Allen JC, Lim YL, Chua SH, Tan SH, Tang MB (2014) Mortality of bullous pemphigoid in Singapore: risk factors and causes of death in 359 patients seen at the National Skin Centre. Br J Dermatol 170:1319–1326. doi:10.1111/bjd.12806

    Article  CAS  PubMed  Google Scholar 

  14. Zhang LM, Wu J, Xiao T, Jin GY, Li JH, Geng L, He CD, Gao XH, Chen HD (2013) Treatment and mortality rate of bullous pemphigoid in China: a hospital-based study. Eur J Dermatol 23:94–98. doi:10.1684/ejd.2012.1906

    CAS  PubMed  Google Scholar 

  15. Gual A, Mascaro JM Jr, Rojas-Farreras S, Guilabert A, Julia M, Iranzo P (2014) Mortality of bullous pemphigoid in the first year after diagnosis: a retrospective study in a Spanish medical centre. J Eur Acad Dermatol Venereol 28:500–506. doi:10.1111/jdv.12065

    Article  CAS  PubMed  Google Scholar 

  16. Cortes B, Khelifa E, Clivaz L, Cazzaniga S, Saurat JH, Naldi L, Borradori L (2012) Mortality rate in bullous pemphigoid: a retrospective monocentric cohort study. Dermatology 225:320–325. doi:10.1159/000345625

    Article  CAS  PubMed  Google Scholar 

  17. Baibergenova AT, Weinstock MA, Shear NH (2012) Mortality from acquired bullous diseases of skin in Canadian adults 2000-2007. Int J Dermatol 51:1325–1328. doi:10.1111/j.1365-4632.2011.05227.x

    Article  PubMed  Google Scholar 

  18. Cortes B, Marazza G, Naldi L, Combescure C, Borradori L (2011) Mortality of bullous pemphigoid in Switzerland: a prospective study. Br J Dermatol 165:368–374. doi:10.1111/j.1365-2133.2011.10413.x

    Article  CAS  PubMed  Google Scholar 

  19. Fichel F, Barbe C, Joly P, Bedane C, Vabres P, Truchetet F, Aubin F, Michel C, Jegou J, Grange F, Antonicelli F, Bernard P (2014) Clinical and immunologic factors associated with bullous pemphigoid relapse during the first year of treatment: a multicenter, prospective study. JAMA Dermatol 150:25–33. doi:10.1001/jamadermatol.2013.5757

    Article  PubMed  Google Scholar 

  20. Rzany B, Partscht K, Jung M et al (2002) Risk factors for lethal outcome in patients with bullous pemphigoid: low serum albumin level, high dosage of glucocorticosteroids, and old age. Arch Dermatol 138:903–908

    Article  PubMed  Google Scholar 

  21. Ren Z, Hsu DY, Brieva J, Silverberg NB, Langan SM, Silverberg JI (2016) Hospitalization, inpatient burden and comorbidities associated with bullous pemphigoid in the U.S.A. Br J Dermatol. 10.1111/bjd.14821

  22. Phoon YW, Fook-Chong SM, Koh HY, Thirumoorthy T, Pang SM, Lee HY (2015) Infectious complications in bullous pemphigoid: an analysis of risk factors. J Am Acad Dermatol 72:834–839. doi:10.1016/j.jaad.2015.01.029

    Article  PubMed  Google Scholar 

  23. Lehman JS, Khunger M, Lohse CM (2013) Infection in autoimmune bullous diseases: a retrospective comparative study. J Dermatol 40:613–619. doi:10.1111/1346-8138.12175

    Article  PubMed  Google Scholar 

  24. Barrick BJ, Lohse CM, Lehman JS (2015) Specific causes of death in patients with bullous pemphigoid as measured by death certificate data: a retrospective cohort study. Int J Dermatol 54:56–61. doi:10.1111/ijd.12243

    Article  PubMed  Google Scholar 

  25. Garcia-Doval I, Conde Taboada A, Cruces Prado MJ (2005) Sepsis associated with dermatologic hospitalization is not the cause of high mortality of bullous pemphigoid in Europe. J Invest Dermatol 124:666–667. doi:10.1111/j.0022-202X.2005.23628.x

    Article  PubMed  Google Scholar 

  26. Robinson ES, Payne AS, Pappas-Taffer L, Feng R, Werth VP (2016) The incidence of herpes zoster in cutaneous lupus erythematosus (CLE), dermatomyositis (DM), pemphigus vulgaris (PV), and bullous pemphigoid (BP). J Am Acad Dermatol 75:42–48. doi:10.1016/j.jaad.2016.02.1153

    Article  PubMed  Google Scholar 

  27. Cugno M, Marzano AV, Bucciarelli P, Balice Y, Cianchini G, Quaglino P, Calzavara Pinton P, Caproni M, Alaibac M, De Simone C, Patrizi A, Cozzani E, Papini M, Tedeschi A, Berti E, Rosendaal FR (2016) Increased risk of venous thromboembolism in patients with bullous pemphigoid. The INVENTEP (INcidence of VENous ThromboEmbolism in bullous Pemphigoid) study. Thromb Haemost 115:193–199. doi:10.1160/th15-04-0309

    Article  PubMed  Google Scholar 

  28. Delgado JC, Turbay D, Yunis EJ, Yunis JJ, Morton ED, Bhol K, Norman R, Alper CA, Good RA, Ahmed R (1996) A common major histocompatibility complex class II allele HLA-DQB1* 0301 is present in clinical variants of pemphigoid. Proc Natl Acad Sci U S A 93:8569–8571

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  29. Setterfield J, Theron J, Vaughan RW, Welsh KI, Mallon E, Wojnarowska F, Challacombe SJ, Black MM (2001) Mucous membrane pemphigoid: HLA-DQB1*0301 is associated with all clinical sites of involvement and may be linked to antibasement membrane IgG production. Br J Dermatol 145:406–414

    Article  CAS  PubMed  Google Scholar 

  30. Zakka LR, Reche P, Ahmed AR (2011) Role of MHC class II genes in the pathogenesis of pemphigoid. Autoimmun Rev 11:40–47. doi:10.1016/j.autrev.2011.07.002

    Article  CAS  PubMed  Google Scholar 

  31. Banfield CC, Wojnarowska F, Allen J, George S, Venning VA, Welsh KI (1998) The association of HLA-DQ7 with bullous pemphigoid is restricted to men. Br J Dermatol 138:1085–1090

    Article  CAS  PubMed  Google Scholar 

  32. Horton R, Wilming L, Rand V, Lovering RC, Bruford EA, Khodiyar VK, Lush MJ, Povey S, Talbot CC Jr, Wright MW, Wain HM, Trowsdale J, Ziegler A, Beck S (2004) Gene map of the extended human MHC. Nat Rev Genet 5:889–899. doi:10.1038/nrg1489

    Article  CAS  PubMed  Google Scholar 

  33. Yancey KB, Egan CA (2000) Pemphigoid: clinical, histologic, immunopathologic, and therapeutic considerations. JAMA 284:350–356

    Article  CAS  PubMed  Google Scholar 

  34. Hirose M, Schilf P, Benoit S, Eming R, Glaser R, Homey B, Kunz M, Nebel A, Peitsch WK, Pfohler C, Sardy M, Schreiber S, Zillikens D, Schmidt E, Ibrahim SM (2015) Polymorphisms in the mitochondrially encoded ATP synthase 8 gene are associated with susceptibility to bullous pemphigoid in the German population. Exp Dermatol 24:715–717. doi:10.1111/exd.12732

    Article  PubMed  Google Scholar 

  35. Cai SC, Allen JC, Lim YL, Tan SH, Tang MB (2015) Association of bullous pemphigoid and malignant neoplasms. JAMA Dermatol 151:665–667. doi:10.1001/jamadermatol.2014.5263

    Article  PubMed  Google Scholar 

  36. Schroeter AL (1987) Pemphigoid and malignancy. Clin Dermatol 5:60–63

    Article  CAS  PubMed  Google Scholar 

  37. Venning VA, Wojnarowska F (1990) The association of bullous pemphigoid and malignant disease: a case control study. Br J Dermatol 123:439–445

    Article  CAS  PubMed  Google Scholar 

  38. Lindelof B, Islam N, Eklund G, Arfors L (1990) Pemphigoid and cancer. Arch Dermatol 126:66–68

    Article  CAS  PubMed  Google Scholar 

  39. Ong E, Goldacre R, Hoang U, Sinclair R, Goldacre M (2014) Associations between bullous pemphigoid and primary malignant cancers: an English national record linkage study, 1999-2011. Arch Dermatol Res 306:75–80. doi:10.1007/s00403-013-1399-5

    Article  PubMed  Google Scholar 

  40. Schulze F, Neumann K, Recke A, Zillikens D, Linder R, Schmidt E (2015) Malignancies in pemphigus and pemphigoid diseases. J Invest Dermatol 135:1445–1447. doi:10.1038/jid.2014.547

    Article  CAS  PubMed  Google Scholar 

  41. Ogawa H, Sakuma M, Morioka S, Kitamura K, Sasai Y, Imamura S, Inaba Y (1995) The incidence of internal malignancies in pemphigus and bullous pemphigoid in Japan. J Dermatol Sci 9:136–141

    Article  CAS  PubMed  Google Scholar 

  42. Schmidt E, della Torre R, Borradori L (2012) Clinical features and practical diagnosis of bullous pemphigoid. Immunol Allergy Clin North Am 32:217–232, v. 10.1016/j.iac.2012.04.002

  43. Borradori L, Joly P (2014) Toward a practical renaming of bullous pemphigoid and all its variants: cutaneous pemphigoid. JAMA Dermatol 150:459. doi:10.1001/jamadermatol.2014.51

    Article  PubMed  Google Scholar 

  44. Schmidt T, Sitaru C, Amber K, Hertl M (2014) BP180- and BP230-specific IgG autoantibodies in pruritic disorders of the elderly: a preclinical stage of bullous pemphigoid? Br J Dermatol 171:212–219. doi:10.1111/bjd.12936

    Article  CAS  PubMed  Google Scholar 

  45. della Torre R, Combescure C, Cortes B, Marazza G, Beltraminelli H, Naldi L, Borradori L (2012) Clinical presentation and diagnostic delay in bullous pemphigoid: a prospective nationwide cohort. Br J Dermatol 167:1111–1117. 10.1111/j.1365-2133.2012.11108.x

  46. Asbrink E, Hovmark A (1981) Clinical variations in bullous pemphigoid with respect to early symptoms. Acta Derm Venereol 61:417–421

    CAS  PubMed  Google Scholar 

  47. Amber KT, Korta DZ, de Feraudy S, Grando SA (2017) Vesiculobullous eruption in a patient receiving psoralen ultraviolet A (PUVA) treatment for prurigo nodules: a case of PUVA-aggravated pemphigoid nodularis. Clin Exp Dermatol. doi:10.1111/ced.13172

  48. Hashimoto T, Ohzono A, Teye K, Numata S, Hiroyasu S, Tsuruta D, Hachiya T, Kuroda K, Hashiguchi M, Kawakami T, Ishii N (2016) Detection of IgE autoantibodies to BP180 and BP230 and their relationship to clinical features in bullous pemphigoid. Br J Dermatol. doi:10.1111/bjd.15114

  49. Di Zenzo G, Della Torre R, Zambruno G, Borradori L (2012) Bullous pemphigoid: from the clinic to the bench. Clin Dermatol 30:3–16. doi:10.1016/j.clindermatol.2011.03.005

    Article  PubMed  Google Scholar 

  50. Jawitz J, Kumar V, Nigra TP, Beutner EH (1984) Vesicular pemphigoid vs dermatitis herpetiformis. J Am Acad Dermatol 10:892–896

    Article  CAS  PubMed  Google Scholar 

  51. Sander HM, Utz MM, Peters MS (1989) Bullous pemphigoid and dermatitis herpetiformis: mixed bullous disease or coexistence of two separate entities? J Cutan Pathol 16:370–374

    Article  CAS  PubMed  Google Scholar 

  52. Bean SF, Michel B, Furey N, Thorne G, Meltzer L (1976) Vesicular pemphigoid. Arch Dermatol 112:1402–1404

    Article  CAS  PubMed  Google Scholar 

  53. Geyer AS, Zillikens D, Skrobek C, Cohen B, Anhalt GJ, Nousari HC (2003) Vesicular pemphigoid in a 16-year-old boy. J Am Acad Dermatol 49:722–724

    Article  PubMed  Google Scholar 

  54. Hayakawa K, Shimizu H, Amagai M, Harada T, Nishikawa T, Yamasaki Y (1989) Vesicular pemphigoid—ultrastructural and immunoelectron microscopic study. Dermatologica 178:213–216

    Article  CAS  PubMed  Google Scholar 

  55. Gruber GG, Owen LG, Callen JP (1980) Vesicular pemphigoid. J Am Acad Dermatol 3:619–622

    Article  CAS  PubMed  Google Scholar 

  56. Inoh Y, Nishikawa T, Hashimoto T (1998) The vesicular pemphigoid phenotype may be related to antibodies to a 200 kDa antigen in the lower lamina lucida. Br J Dermatol 139:738–739

    Article  CAS  PubMed  Google Scholar 

  57. Zaraa I, Mahfoudh A, Sellami MK, Chelly I, El Euch D, Zitouna M, Mokni M, Makni S, Ben Osman A (2013) Lichen planus pemphigoides: four new cases and a review of the literature. Int J Dermatol 52:406–412. doi:10.1111/j.1365-4632.2012.05693.x

    Article  PubMed  Google Scholar 

  58. Cohen DM, Ben-Amitai D, Feinmesser M, Zvulunov A (2009) Childhood lichen planus pemphigoides: a case report and review of the literature. Pediatr Dermatol 26:569–574. doi:10.1111/j.1525-1470.2009.00988.x

    Article  PubMed  Google Scholar 

  59. Joly P, Tanasescu S, Wolkenstein P, Bocquet H, Gilbert D, Thomine E, Wechsler J, Roujeau JC, Revuz J, Tron F, Lauret P (1998) Lichenoid erythrodermic bullous pemphigoid of the African patient. J Am Acad Dermatol 39:691–697

    Article  CAS  PubMed  Google Scholar 

  60. Korman NJ, Woods SG (1995) Erythrodermic bullous pemphigoid is a clinical variant of bullous pemphigoid. Br J Dermatol 133:967–971

    Article  CAS  PubMed  Google Scholar 

  61. Wallach D, Cottenot F (1982) Erythrodermic bullous pemphigoid or erythrodermic psoriasis and bullous pemphigoid? J Am Acad Dermatol 7:800

    Article  CAS  PubMed  Google Scholar 

  62. Alonso-Llamazares J, Dietrich SM, Gibson LE (1998) Bullous pemphigoid presenting as exfoliative erythroderma. J Am Acad Dermatol 39:827–830

    Article  CAS  PubMed  Google Scholar 

  63. Kim J, Chavel S, Girardi M, McNiff JM (2008) Pemphigoid vegetans: a case report and review of the literature. J Cutan Pathol 35:1144–1147

    Article  PubMed  Google Scholar 

  64. Chan LS, Dorman MA, Agha A, Suzuki T, Cooper KD, Hashimoto K (1993) Pemphigoid vegetans represents a bullous pemphigoid variant. Patient's IgG autoantibodies identify the major bullous pemphigoid antigen J Am Acad Dermatol 28:331–335

    CAS  PubMed  Google Scholar 

  65. Schnyder MU (1969) Seborrheic pemphigoid. A new nosologic entity? Bull Soc Fr Dermatol Syphiligr 76:320

    CAS  PubMed  Google Scholar 

  66. Castro-Fores LL, Cruz JM, Jamora MJ, Canlas-Estrella KM (2016) Seborrheic pemphigoid. JAAD Case Rep 2:80–83. doi:10.1016/j.jdcr.2015.12.010

    Article  PubMed Central  PubMed  Google Scholar 

  67. Errichetti E, Stinco G (2014) Seborrheic pemphigoid 2014:768217. doi:10.1155/2014/768217

    Google Scholar 

  68. Cordel N, Courville P, Martel P, Musette P, Joly P (2002) Extensive erosive bullous pemphigoid: an atypical and serious clinical variant. Br J Dermatol 146:537–539

    Article  CAS  PubMed  Google Scholar 

  69. Marovt M, El Shabrawi-Caelen L (2015) Purpuric bullous pemphigoid. Am J Dermatopathol 37:e18–e20. doi:10.1097/dad.0000000000000057

    Article  PubMed  Google Scholar 

  70. Schwieger-Briel A, Moellmann C, Mattulat B, Schauer F, Kiritsi D, Schmidt E, Sitaru C, Ott H, Kern JS (2014) Bullous pemphigoid in infants: characteristics, diagnosis and treatment. Orphanet J Rare Dis 9:185. doi:10.1186/s13023-014-0185-6

    Article  PubMed Central  PubMed  Google Scholar 

  71. Pietkiewicz P, Gornowicz-Porowska J, Bowszyc-Dmochowska M, Bartkiewicz P, Dmochowski M (2016) Bullous pemphigoid and neurodegenerative diseases: a study in a setting of a Central European university dermatology department. Aging Clin Exp Res 28:659–663. doi:10.1007/s40520-015-0459-4

    Article  PubMed  Google Scholar 

  72. Chen YJ, Wu CY, Lin MW, Chen TJ, Liao KK, Chen YC, Hwang CY, Chu SY, Chen CC, Lee DD, Chang YT, Wang WJ, Liu HN (2011) Comorbidity profiles among patients with bullous pemphigoid: a nationwide population-based study. Br J Dermatol 165:593–599. doi:10.1111/j.1365-2133.2011.10386.x

    Article  CAS  PubMed  Google Scholar 

  73. Salomon RJ, Briggaman RA, Wernikoff SY, Kayne AL (1987) Localized bullous pemphigoid. A mimic of acute contact dermatitis. Arch Dermatol 123:389–392

    Article  CAS  PubMed  Google Scholar 

  74. Batalla A, Peon G, De la Torre C (2011) Localized bullous pemphigoid at urostomy site. Indian J Dermatol Venereol Leprol 77:625. doi:10.4103/0378-6323.84067

    Article  PubMed  Google Scholar 

  75. Egan CA, Florell SR, Zone JJ (1999) Localized bullous pemphigoid in a patient with B-cell lymphoma. South Med J 92:1220–1222

    Article  CAS  PubMed  Google Scholar 

  76. Nguyen T, Kwan JM, Ahmed AR (2014) Relationship between radiation therapy and bullous pemphigoid. Dermatology 229:88–96. doi:10.1159/000362208

    Article  CAS  PubMed  Google Scholar 

  77. Mul VE, van Geest AJ, Pijls-Johannesma MC, Theys J, Verschueren TA, Jager JJ, Lambin P, Baumert BG (2007) Radiation-induced bullous pemphigoid: a systematic review of an unusual radiation side effect. Radiother Oncol 82:5–9. doi:10.1016/j.radonc.2006.11.014

    Article  PubMed  Google Scholar 

  78. Sears A, Wee JS, Misch K, Natkunarajah J (2012) Bullous pemphigoid induced by radiotherapy recurring on rechallenge. Clin Exp Dermatol 37:916–917. doi:10.1111/j.1365-2230.2012.04402.x

    Article  CAS  PubMed  Google Scholar 

  79. Tsuruta D, Nishikawa T, Yamagami J, Hashimoto T (2012) Unilateral bullous pemphigoid without erythema and eosinophil infiltration in a hemiplegic patient. J Dermatol 39:787–789. doi:10.1111/j.1346-8138.2012.01562.x

    Article  CAS  PubMed  Google Scholar 

  80. Tay YK, Cheong WK (1993) An unusual case of localised pemphigoid. Ann Acad Med Singap 22:937–938

    CAS  PubMed  Google Scholar 

  81. Bunker CB, Brown E (1993) Unilateral bullous pemphigoid in a hemiplegic patient. Br J Dermatol 129:502

    Article  CAS  PubMed  Google Scholar 

  82. Long CC, Lever LR, Marks R (1992) Unilateral bullous pemphigoid in a hemiplegic patient. Br J Dermatol 126:614–616

    Article  CAS  PubMed  Google Scholar 

  83. Foureur N, Descamps V, Lebrun-Vignes B, Picard-Dahan C, Grossin M, Belaich S, Crickx B (2001) Bullous pemphigoid in a leg affected with hemiparesia: a possible relation of neurological diseases with bullous pemphigoid? Eur J Dermatol 11:230–233

    CAS  PubMed  Google Scholar 

  84. Reilly GD, Boulton AJ, Harrington CI (1983) Stump pemphigoid: a new complication of the amputee. Br Med J (Clin Res Ed) 287:875–876

    Article  CAS  Google Scholar 

  85. Brodell RT, Korman NJ (1996) Stump pemphigoid. Cutis 57:245–246

    CAS  PubMed  Google Scholar 

  86. Muramatsu T, Iida T, Shirai T (1991) Antibasement membrane zone antibodies in localized pretibial pemphigoid. Int J Dermatol 30:422–424

    Article  CAS  PubMed  Google Scholar 

  87. Borradori L, Prost C, Wolkenstein P, Bernard P, Baccard M, Morel P (1992) Localized pretibial pemphigoid and pemphigoid nodularis. J Am Acad Dermatol 27:863–867

    Article  CAS  PubMed  Google Scholar 

  88. Kakurai M, Demitsu T, Azuma R, Yamada T, Suzuki M, Yoneda K, Ishii N, Hashimoto T (2007) Localized pemphigoid (pretibial type) with IgG antibody to BP180 NC16a domain successfully treated with minocycline and topical corticosteroid. Clin Exp Dermatol 32:759–761. doi:10.1111/j.1365-2230.2007.02527.x

    Article  CAS  PubMed  Google Scholar 

  89. Fisler RE, Saeb M, Liang MG, Howard RM, McKee PH (2003) Childhood bullous pemphigoid: a clinicopathologic study and review of the literature. Am J Dermatopathol 25:183–189

    Article  PubMed  Google Scholar 

  90. Saad RW, Domloge-Hultsch N, Yancey KB, Benson PM, James WD (1992) Childhood localized vulvar pemphigoid is a true variant of bullous pemphigoid. Arch Dermatol 128:807–810

    Article  CAS  PubMed  Google Scholar 

  91. Farrell AM, Kirtschig G, Dalziel KL, Allen J, Dootson G, Edwards S, Wojnarowska F (1999) Childhood vulval pemphigoid: a clinical and immunopathological study of five patients. Br J Dermatol 140:308–312

    Article  CAS  PubMed  Google Scholar 

  92. Schmidt E, Benoit S, Brocker EB (2009) Bullous pemphigoid with localized umbilical involvement. Acta Derm Venereol 89:419–420. doi:10.2340/00015555-0644

    Article  PubMed  Google Scholar 

  93. Gambichler T, Segert H, Hoxtermann S, Schmitz L, Altmeyer P, Teegen B (2015) Neurological disorders in patients with bullous pemphigoid: clinical and experimental investigations. J Eur Acad Dermatol Venereol 29:1758–1762. doi:10.1111/jdv.12995

    Article  CAS  PubMed  Google Scholar 

  94. Teixeira VB, Cabral R, Brites MM, Vieira R, Figueiredo A (2014) Bullous pemphigoid and comorbidities: a case-control study in Portuguese patients. An Bras Dermatol 89:274–278

    Article  PubMed Central  PubMed  Google Scholar 

  95. Jedlickova H, Hlubinka M, Pavlik T, Semradova V, Budinska E, Vlasin Z (2010) Bullous pemphigoid and internal diseases—a case-control study. Eur J Dermatol 20:96–101. doi:10.1684/ejd.2010.0805

    PubMed  Google Scholar 

  96. Taghipour K, Chi CC, Vincent A, Groves RW, Venning V, Wojnarowska F (2010) The association of bullous pemphigoid with cerebrovascular disease and dementia: a case-control study. Arch Dermatol 146:1251–1254. doi:10.1001/archdermatol.2010.322

    Article  PubMed  Google Scholar 

  97. Forsti AK, Jokelainen J, Ansakorpi H, Seppanen A, Majamaa K, Timonen M, Tasanen K (2016) Psychiatric and neurological disorders are associated with bullous pemphigoid—a nationwide Finnish Care Register study. Sci Rep 6:37125. doi:10.1038/srep37125

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  98. Lai YC, Yew YW, Lambert WC (2016) Bullous pemphigoid and its association with neurological diseases: a systematic review and meta-analysis. J Eur Acad Dermatol Venereol. doi:10.1111/jdv.13660

  99. Brick KE, Weaver CH, Savica R, Lohse CM, Pittelkow MR, Boeve BF, Gibson LE, Camilleri MJ, Wieland CN (2014) A population-based study of the association between bullous pemphigoid and neurologic disorders. J Am Acad Dermatol 71:1191–1197. doi:10.1016/j.jaad.2014.07.052

    Article  PubMed Central  PubMed  Google Scholar 

  100. Tarazona MJ, Mota AN, Gripp AC, Unterstell N, Bressan AL (2015) Bullous pemphigoid and neurological disease: statistics from a dermatology service. An Bras Dermatol 90:280–282. doi:10.1590/abd1806-4841.20153334

    Article  PubMed Central  PubMed  Google Scholar 

  101. Langan SM, Groves RW, West J (2011) The relationship between neurological disease and bullous pemphigoid: a population-based case-control study. J Invest Dermatol 131:631–636. doi:10.1038/jid.2010.357

    Article  CAS  PubMed  Google Scholar 

  102. Daneshpazhooh M, Khorassani J, Balighi K, Ghandi N, Mahmoudi H, Tohidinik H, Hamzelou S, Chams-Davatchi C (2016) Neurological diseases and bullous pemphigoid: a case-control study in Iranian patients. Indian J Dermatol Venereol Leprol. 10.4103/0378–6323.191132

  103. Bastuji-Garin S, Joly P, Lemordant P, Sparsa A, Bedane C, Delaporte E, Roujeau JC, Bernard P, Guillaume JC, Ingen-Housz-Oro S, Maillard H, Pauwels C, Picard-Dahan C, Dutronc Y, Richard MA (2011) Risk factors for bullous pemphigoid in the elderly: a prospective case-control study. J Invest Dermatol 131:637–643. doi:10.1038/jid.2010.301

    Article  CAS  PubMed  Google Scholar 

  104. Chevalier V, Barbe C, Reguiai Z, Plee J, Grange F, Bernard P (2016) Impact of neurological diseases on the prognosis of bullous pemphigoid: a retrospective study of 178 patients. Ann Dermatol Venereol 143:179–186. doi:10.1016/j.annder.2015.12.016

    Article  CAS  PubMed  Google Scholar 

  105. Cordel N, Chosidow O, Hellot MF, Delaporte E, Lok C, Vaillant L, Bernard P, D'Incan M, Roujeau JC, Joly P (2007) Neurological disorders in patients with bullous pemphigoid. Dermatology 215:187–191. doi:10.1159/000106574

    Article  PubMed  Google Scholar 

  106. Seppanen A, Autio-Harmainen H, Alafuzoff I, Sarkioja T, Veijola J, Hurskainen T, Bruckner-Tuderman L, Tasanen K, Majamaa K (2006) Collagen XVII is expressed in human CNS neurons. Matrix Biol 25:185–188. doi:10.1016/j.matbio.2005.11.004

    Article  PubMed  CAS  Google Scholar 

  107. Claudepierre T, Manglapus MK, Marengi N, Radner S, Champliaud MF, Tasanen K, Bruckner-Tuderman L, Hunter DD, Brunken WJ (2005) Collagen XVII and BPAG1 expression in the retina: evidence for an anchoring complex in the central nervous system. J Comp Neurol 487:190–203. doi:10.1002/cne.20549

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  108. Seppanen A, Suuronen T, Hofmann SC, Majamaa K, Alafuzoff I (2007) Distribution of collagen XVII in the human brain. Brain Res 1158:50–56. doi:10.1016/j.brainres.2007.04.073

    Article  PubMed  CAS  Google Scholar 

  109. Amber KT, Zikry J, Hertl M (2017) A multi-hit hypothesis of bullous pemphigoid and associated neurological disease: is HLA-DQB1*03:01, a potential link between immune privileged antigen exposure and epitope spreading? Hla. 10.1111/tan.12960

  110. Messingham KA, Aust S, Helfenberger J, Parker KL, Schultz S, McKillip J, Narayanan NS, Fairley JA (2016) Autoantibodies to collagen XVII are present in Parkinson's disease and localize to tyrosine-hydroxylase positive neurons. J Invest Dermatol 136:721–723. doi:10.1016/j.jid.2015.12.005

    Article  CAS  PubMed  Google Scholar 

  111. Recke A, Oei A, Hubner F, Fechner K, Graf J, Hagenah J, May C, Woitalla D, Salmen A, Zillikens D, Gold R, Schlumberger W, Schmidt E (2016) Parkinson disease and multiple sclerosis are not associated with autoantibodies against structural proteins of the dermal-epidermal junction. Br J Dermatol 175:407–409. doi:10.1111/bjd.14538

    Article  CAS  PubMed  Google Scholar 

  112. Moutsianas L, Jostins L, Beecham AH et al (2015) Class II HLA interactions modulate genetic risk for multiple sclerosis. Nat Genet 47:1107–1113. doi:10.1038/ng.3395

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  113. Weinshenker BG, Santrach P, Bissonet AS, McDonnell SK, Schaid D, Moore SB, Rodriguez M (1998) Major histocompatibility complex class II alleles and the course and outcome of MS: a population-based study. Neurology 51:742–747

    Article  CAS  PubMed  Google Scholar 

  114. Marrosu MG, Murru MR, Costa G, Murru R, Muntoni F, Cucca F (1998) DRB1-DQA1-DQB1 loci and multiple sclerosis predisposition in the Sardinian population. Hum Mol Genet 7:1235–1237

    Article  CAS  PubMed  Google Scholar 

  115. Fernandez O, A RA, Pinto-Medel MJ, Mendibe MM, Acosta N, Oliver B, Guerrero M, Papais-Alvarenga M, Fernandez-Sanchez V, Leyva L (2009) HLA class II alleles in patients with multiple sclerosis in the Biscay province (Basque Country, Spain). J Neurol 256:1977–1988. 10.1007/s00415-009-5223-2

  116. Al-Shammri S, Nelson RF, Al-Muzairi I, Akanji AO (2004) HLA determinants of susceptibility to multiple sclerosis in an Arabian Gulf population. Mult Scler 10:381–386

    Article  CAS  PubMed  Google Scholar 

  117. Oh HH, Kwon SH, Kim CW, Choe BH, Ko CW, Jung HD, Suh JS, Lee JH (2004) Molecular analysis of HLA class II-associated susceptibility to neuroinflammatory diseases in Korean children. J Korean Med Sci 19:426–430. doi:10.3346/jkms.2004.19.3.426

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  118. Kouris A, Platsidaki E, Christodoulou C, Armyra K, Korkoliakou P, Stefanaki C, Tsatovidou R, Rigopoulos D, Kontochristopoulos G (2016) Quality of life, depression, anxiety and loneliness in patients with bullous pemphigoid. A case control study. An Bras Dermatol 91:601–603. doi:10.1590/abd1806-4841.20164935

    Article  PubMed Central  PubMed  Google Scholar 

  119. Lloyd-Lavery A, Chi CC, Wojnarowska F, Taghipour K (2013) The associations between bullous pemphigoid and drug use: a UK case-control study. JAMA Dermatol 149:58–62. doi:10.1001/2013.jamadermatol.376

    Article  PubMed  Google Scholar 

  120. Bastuji-Garin S, Joly P, Picard-Dahan C, Bernard P, Vaillant L, Pauwels C, Salagnac V, Lok C, Roujeau JC (1996) Drugs associated with bullous pemphigoid. A case-control study. Arch Dermatol 132:272–276

    Article  CAS  PubMed  Google Scholar 

  121. CW XT, Pang Y, Sim B, Thirumoorthy T, Pang SM, Lee HY (2016) The association between drugs and bullous pemphigoid. Br J Dermatol 10.1111/bjd.15195

  122. Bene J, Moulis G, Bennani I, Auffret M, Coupe P, Babai S, Hillaire-Buys D, Micallef J, Gautier S (2016) Bullous pemphigoid and dipeptidyl peptidase IV inhibitors: a case-noncase study in the French pharmacovigilance database. Br J Dermatol 175:296–301. doi:10.1111/bjd.14601

    Article  CAS  PubMed  Google Scholar 

  123. Naidoo J, Schindler K, Querfeld C, Busam K, Cunningham J, Page DB, Postow MA, Weinstein A, Lucas AS, Ciccolini KT, Quigley EA, Lesokhin AM, Paik PK, Chaft JE, Segal NH, D'Angelo SP, Dickson MA, Wolchok JD, Lacouture ME (2016) Autoimmune bullous skin disorders with immune checkpoint inhibitors targeting PD-1 and PD-L1. Cancer Immunol Res 4:383–389. doi:10.1158/2326-6066.cir-15-0123

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  124. Jour G, Glitza IC, Ellis RM, Torres-Cabala CA, Tetzlaff MT, Li JY, Nagarajan P, Huen A, Aung PP, Ivan D, Drucker CR, Prieto VG, Rapini RP, Patel A, Curry JL (2016) Autoimmune dermatologic toxicities from immune checkpoint blockade with anti-PD-1 antibody therapy: a report on bullous skin eruptions. J Cutan Pathol 43:688–696. doi:10.1111/cup.12717

    Article  PubMed  Google Scholar 

  125. Mochel MC, Ming ME, Imadojemu S, Gangadhar TC, Schuchter LM, Elenitsas R, Payne AS, Chu EY (2016) Cutaneous autoimmune effects in the setting of therapeutic immune checkpoint inhibition for metastatic melanoma. J Cutan Pathol 43:787–791. doi:10.1111/cup.12735

    Article  PubMed  Google Scholar 

  126. Amber KT, Panganiban CM, Korta D, Feraudy S, Kelly KM, Grando SA (2016) A case report of bullous pemphigoid associated with a melanoma and review of the literature. Melanoma Res. doi:10.1097/cmr.0000000000000307

  127. Robinson JK, Baughman RD, Provost TT (1978) Bullous pemphigoid induced by PUVA therapy. Is this the aetiology of the acral bullae produced during PUVA treatment? Br J Dermatol 99:709–713

    Article  CAS  PubMed  Google Scholar 

  128. George PM (1996) Bullous pemphigoid possibly induced by psoralen plus ultraviolet a therapy. Photodermatol Photoimmunol Photomed 11:185–187

    Article  CAS  PubMed  Google Scholar 

  129. Barnadas MA, Gilaberte M, Pujol R, Agusti M, Gelpi C, Alomar A (2006) Bullous pemphigoid in a patient with psoriasis during the course of PUVA therapy: study by ELISA test. Int J Dermatol 45:1089–1092. doi:10.1111/j.1365-4632.2004.02517.x

    Article  PubMed  Google Scholar 

  130. Riyaz N, Nasir N, Bindu V, Sasidharanpillai S (2014) Bullous pemphigoid induced by topical PUVASOL. Indian J Dermatol Venereol Leprol 80:363–364. doi:10.4103/0378-6323.136936

    Article  PubMed  Google Scholar 

  131. Kuramoto N, Kishimoto S, Shibagaki R, Yasuno H (2000) PUVA-induced lichen planus pemphigoides. Br J Dermatol 142:509–512

    Article  CAS  PubMed  Google Scholar 

  132. Ohata C, Ishii N, Koga H, Fukuda S, Tateishi C, Tsuruta D, Furumura M, Hashimoto T (2015) Coexistence of autoimmune bullous diseases (AIBDs) and psoriasis: a series of 145 cases. J Am Acad Dermatol 73:50–55. doi:10.1016/j.jaad.2015.03.016

    Article  PubMed  Google Scholar 

  133. Kirtschig G, Chow ET, Venning VA, Wojnarowska FT (1996) Acquired subepidermal bullous diseases associated with psoriasis: a clinical, immunopathological and immunogenetic study. Br J Dermatol 135:738–745

    Article  CAS  PubMed  Google Scholar 

  134. Chung SD, Lin HC, Wang KH (2014) Increased risk of pemphigoid following scabies: a population-based matched-cohort study. J Eur Acad Dermatol Venereol 28:558–564. doi:10.1111/jdv.12132

    Article  PubMed  Google Scholar 

  135. Joly P, Roujeau JC, Benichou J et al (2009) A comparison of two regimens of topical corticosteroids in the treatment of patients with bullous pemphigoid: a multicenter randomized study. J Invest Dermatol 129:1681–1687. doi:10.1038/jid.2008.412

    Article  CAS  PubMed  Google Scholar 

  136. Joly P, Roujeau JC, Benichou J, Picard C, Dreno B, Delaporte E, Vaillant L, D'Incan M, Plantin P, Bedane C, Young P, Bernard P (2002) A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med 346:321–327. doi:10.1056/NEJMoa011592

    Article  CAS  PubMed  Google Scholar 

  137. Schneiderbauer R, Martinache S, Engstner M, Enk AH, Hadaschik EN (2016) Correlation of autoantibodies against BP180/BP230 in response to topical corticosteroids in patients with bullous pemphigoid. Dermatol Ther. doi:10.1111/dth.12403

  138. Eming R, Sticherling M, Hofmann SC, Hunzelmann N, Kern JS, Kramer H, Pfeiffer C, Schuster V, Zillikens D, Goebeler M, Hertl M, Nast A, Orzechowski HD, Sardy M, Schmidt E, Sitaru C, Sporbeck B, Worm M (2015) S2k guidelines for the treatment of pemphigus vulgaris/foliaceus and bullous pemphigoid. J Dtsch Dermatol Ges 13:833–844. doi:10.1111/ddg.12606

    PubMed  Google Scholar 

  139. Goon AT, Tan SH, Khoo LS, Tan T (2000) Tetracycline and nicotinamide for the treatment of bullous pemphigoid: our experience in Singapore. Singap Med J 41:327–330

    CAS  Google Scholar 

  140. Hornschuh B, Hamm H, Wever S, Hashimoto T, Schroder U, Brocker EB, Zillikens D (1997) Treatment of 16 patients with bullous pemphigoid with oral tetracycline and niacinamide and topical clobetasol. J Am Acad Dermatol 36:101–103

    Article  CAS  PubMed  Google Scholar 

  141. Fivenson DP, Breneman DL, Rosen GB, Hersh CS, Cardone S, Mutasim D (1994) Nicotinamide and tetracycline therapy of bullous pemphigoid. Arch Dermatol 130:753–758

    Article  CAS  PubMed  Google Scholar 

  142. Thomas I, Khorenian S, Arbesfeld DM (1993) Treatment of generalized bullous pemphigoid with oral tetracycline. J Am Acad Dermatol 28:74–77

    Article  CAS  PubMed  Google Scholar 

  143. Kolbach DN, Remme JJ, Bos WH, Jonkman MF, De Jong MC, Pas HH, van der Meer JB (1995) Bullous pemphigoid successfully controlled by tetracycline and nicotinamide. Br J Dermatol 133:88–90

    Article  CAS  PubMed  Google Scholar 

  144. Williams HC, Wojnarowska F, Kirtschig G, Mason J, Godec TR, Schmidt E, Chalmers JR, Childs M, Walton S, Harman K, Chapman A, Whitham D, Nunn AJ (2017) Doxycycline versus prednisolone as an initial treatment strategy for bullous pemphigoid: a pragmatic, non-inferiority, randomised controlled trial. Lancet. doi:10.1016/s0140-6736(17)30560-3

  145. Waisbourd-Zinman O, Ben-Amitai D, Cohen AD, Feinmesser M, Mimouni D, Adir-Shani A, Zlotkin M, Zvulunov A (2008) Bullous pemphigoid in infancy: clinical and epidemiologic characteristics. J Am Acad Dermatol 58:41–48. doi:10.1016/j.jaad.2007.08.010

    Article  PubMed  Google Scholar 

  146. Cho YT, Chu CY, Wang LF (2015) First-line combination therapy with rituximab and corticosteroids provides a high complete remission rate in moderate-to-severe bullous pemphigoid. Br J Dermatol 173:302–304. doi:10.1111/bjd.13633

    Article  CAS  PubMed  Google Scholar 

  147. Shetty S, Ahmed AR (2013) Treatment of bullous pemphigoid with rituximab: critical analysis of the current literature. J Drugs Dermatol 12:672–677

    CAS  PubMed  Google Scholar 

  148. Ahmed AR, Shetty S, Kaveri S, Spigelman ZS (2016) Treatment of recalcitrant bullous pemphigoid (BP) with a novel protocol: a retrospective study with a 6-year follow-up. J Am Acad Dermatol 74(700–708):e703. doi:10.1016/j.jaad.2015.11.030

    Google Scholar 

  149. Ahmed AR (2001) Intravenous immunoglobulin therapy for patients with bullous pemphigoid unresponsive to conventional immunosuppressive treatment. J Am Acad Dermatol 45:825–835. doi:10.1067/mjd.2001.116337

    Article  CAS  PubMed  Google Scholar 

  150. Amagai M, Ikeda S, Hashimoto T et al (2016) A randomized double-blind trial of intravenous immunoglobulin for bullous pemphigoid. J Dermatol Sci. doi:10.1016/j.jdermsci.2016.11.003

  151. Tekin B, Yucelten AD (2015) Infantile bullous pemphigoid treated using intravenous immunoglobulin: case report and review of the literature. Pediatr Dermatol 32:723–726. doi:10.1111/pde.12635

    Article  PubMed  Google Scholar 

  152. Gurcan HM, Ahmed AR (2007) Frequency of adverse events associated with intravenous immunoglobulin therapy in patients with pemphigus or pemphigoid. Ann Pharmacother 41:1604–1610. doi:10.1345/aph.1K198

    Article  CAS  PubMed  Google Scholar 

  153. van Beek N, Luttmann N, Huebner F, Recke A, Karl I, Schulze FS, Zillikens D, Schmidt E (2017) Correlation of serum levels of IgE autoantibodies against BP180 with bullous pemphigoid disease activity. JAMA Dermatol 153:30–38. doi:10.1001/jamadermatol.2016.3357

    Article  PubMed  Google Scholar 

  154. Yu KK, Crew AB, Messingham KA, Fairley JA, Woodley DT (2014) Omalizumab therapy for bullous pemphigoid. J Am Acad Dermatol 71:468–474. doi:10.1016/j.jaad.2014.04.053

    Article  CAS  PubMed  Google Scholar 

  155. Balakirski G, Alkhateeb A, Merk HF, Leverkus M, Megahed M (2016) Successful treatment of bullous pemphigoid with omalizumab as corticosteroid-sparing agent: report of two cases and review of literature. J Eur Acad Dermatol Venereol 30:1778–1782. doi:10.1111/jdv.13758

    Article  CAS  PubMed  Google Scholar 

  156. Yalcin AD, Genc GE, Celik B, Gumuslu S (2014) Anti-IgE monoclonal antibody (omalizumab) is effective in treating bullous pemphigoid and its effects on soluble CD200. Clin Lab 60:523–524

    PubMed  Google Scholar 

  157. Gonul MZ, Keseroglu HO, Ergin C, Ozcan I, Erdem O (2016) Bullous pemphigoid successfully treated with omalizumab. Indian J Dermatol Venereol Leprol 82:577–579. 10.4103/0378–6323.183628

  158. Mersmann M, Dworschak J, Ebermann K, Komorowski L, Schlumberger W, Stocker W, Zillikens D, Probst C, Schmidt E (2016) Immunoadsorber for specific apheresis of autoantibodies in the treatment of bullous pemphigoid. Arch Dermatol Res 308:31–38. doi:10.1007/s00403-015-1606-7

    Article  CAS  PubMed  Google Scholar 

  159. Kasperkiewicz M, Schulze F, Meier M, van Beek N, Nitschke M, Zillikens D, Schmidt E (2014) Treatment of bullous pemphigoid with adjuvant immunoadsorption: a case series. J Am Acad Dermatol 71:1018–1020. doi:10.1016/j.jaad.2014.06.014

    Article  PubMed  Google Scholar 

  160. Kolesnik M, Becker E, Reinhold D, Ambach A, Heim MU, Gollnick H, Bonnekoh B (2014) Treatment of severe autoimmune blistering skin diseases with combination of protein a immunoadsorption and rituximab: a protocol without initial high dose or pulse steroid medication. J Eur Acad Dermatol Venereol 28:771–780. doi:10.1111/jdv.12175

    Article  CAS  PubMed  Google Scholar 

  161. de Graauw E, Sitaru C, Horn M, Borradori L, Yousefi S, Simon HU, Simon D (2017) Evidence for a role of eosinophils in blister formation in bullous pemphigoid. Allergy. doi:10.1111/all.13131

  162. Lipozencic J, Ljubojevic S, Bukvic-Mokos Z (2012) Pemphigoid gestationis. Clin Dermatol 30:51–55. doi:10.1016/j.clindermatol.2011.03.009

    Article  PubMed  Google Scholar 

  163. Shornick JK, Bangert JL, Freeman RG, Gilliam JN (1983) Herpes gestationis: clinical and histologic features of twenty-eight cases. J Am Acad Dermatol 8:214–224

    Article  CAS  PubMed  Google Scholar 

  164. Semkova K, Black M (2009) Pemphigoid gestationis: current insights into pathogenesis and treatment. Eur J Obstet Gynecol Reprod Biol 145:138–144. doi:10.1016/j.ejogrb.2009.05.012

    Article  PubMed  Google Scholar 

  165. Shornick JK, Jenkins RE, Artlett CM, Briggs DC, Welsh KI, Kelly SE, Garvey MP, Black MM (1995) Class II MHC typing in pemphigoid gestationis. Clin Exp Dermatol 20:123–126

    Article  CAS  PubMed  Google Scholar 

  166. Jenkins RE, Hern S, Black MM (1999) Clinical features and management of 87 patients with pemphigoid gestationis. Clin Exp Dermatol 24:255–259

    Article  CAS  PubMed  Google Scholar 

  167. Holmes RC, Black MM, Jurecka W, Dann J, James DC, Timlin D, Bhogal B (1983) Clues to the aetiology and pathogenesis of herpes gestationis. Br J Dermatol 109:131–139

    Article  CAS  PubMed  Google Scholar 

  168. Al-Saif F, Elisa A, Al-Homidy A, Al-Ageel A, Al-Mubarak M (2016) Retrospective analysis of pemphigoid gestationis in 32 Saudi patients—clinicopathological features and a literature review. J Reprod Immunol 116:42–45. doi:10.1016/j.jri.2016.04.286

    Article  CAS  PubMed  Google Scholar 

  169. Tani N, Kimura Y, Koga H, Kawakami T, Ohata C, Ishii N, Hashimoto T (2015) Clinical and immunological profiles of 25 patients with pemphigoid gestationis. Br J Dermatol 172:120–129. doi:10.1111/bjd.13374

    Article  CAS  PubMed  Google Scholar 

  170. Roger D, Vaillant L, Fignon A, Pierre F, Bacq Y, Brechot JF, Grangeponte MC, Lorette G (1994) Specific pruritic diseases of pregnancy. A prospective study of 3192 pregnant women. Arch Dermatol 130:734–739

    Article  CAS  PubMed  Google Scholar 

  171. Jenkins RE, Jones SA, Black MM (1996) Conversion of pemphigoid gestationis to bullous pemphigoid—two refractory cases highlighting this association. Br J Dermatol 135:595–598

    Article  CAS  PubMed  Google Scholar 

  172. Chi CC, Wang SH, Wojnarowska F, Kirtschig G, Davies E, Bennett C (2015) Safety of topical corticosteroids in pregnancy. Cochrane Database Syst Rev:Cd007346. 10.1002/14651858.CD007346.pub3

  173. Gotestam Skorpen C, Hoeltzenbein M, Tincani A, Fischer-Betz R, Elefant E, Chambers C, da Silva J (2016) The EULAR points to consider for use of antirheumatic drugs before pregnancy, and during pregnancy and lactation. 75:795-810. 10.1136/annrheumdis-2015-208840

  174. Benediktsson R, Calder AA, Edwards CR, Seckl JR (1997) Placental 11 beta-hydroxysteroid dehydrogenase: a key regulator of fetal glucocorticoid exposure. Clin Endocrinol 46:161–166

    Article  CAS  Google Scholar 

  175. Doiron P, Pratt M (2010) Antepartum intravenous immunoglobulin therapy in refractory pemphigoid gestationis: case report and literature review. J Cutan Med Surg 14:189–192

    Article  PubMed  Google Scholar 

  176. Tourte M, Brunet-Possenti F, Mignot S, Gavard L, Descamps V (2016) Pemphigoid gestationis: a successful preventive treatment by rituximab. J Eur Acad Dermatol Venereol. doi:10.1111/jdv.13962

  177. Chakravarty EF, Murray ER, Kelman A, Farmer P (2011) Pregnancy outcomes after maternal exposure to rituximab. Blood 117:1499–1506. doi:10.1182/blood-2010-07-295444

    Article  CAS  PubMed  Google Scholar 

  178. Zillikens D, Kawahara Y, Ishiko A, Shimizu H, Mayer J, Rank CV, Liu Z, Giudice GJ, Tran HH, Marinkovich MP, Brocker EB, Hashimoto T (1996) A novel subepidermal blistering disease with autoantibodies to a 200-kDa antigen of the basement membrane zone. J Invest Dermatol 106:1333–1338

    Article  CAS  PubMed  Google Scholar 

  179. Chen KR, Shimizu S, Miyakawa S, Ishiko A, Shimizu H, Hashimoto T (1996) Coexistence of psoriasis and an unusual IgG-mediated subepidermal bullous dermatosis: identification of a novel 200-kDa lower lamina lucida target antigen. Br J Dermatol 134:340–346

    Article  CAS  PubMed  Google Scholar 

  180. Dainichi T, Kurono S, Ohyama B, Ishii N, Sanzen N, Hayashi M, Shimono C, Taniguchi Y, Koga H, Karashima T, Yasumoto S, Zillikens D, Sekiguchi K, Hashimoto T (2009) Anti-laminin gamma-1 pemphigoid. Proc Natl Acad Sci U S A 106:2800–2805

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  181. Groth S, Recke A, Vafia K, Ludwig RJ, Hashimoto T, Zillikens D, Schmidt E (2011) Development of a simple enzyme-linked immunosorbent assay for the detection of autoantibodies in anti-p200 pemphigoid. Br J Dermatol 164:76–82. doi:10.1111/j.1365-2133.2010.10056.x

    Article  CAS  PubMed  Google Scholar 

  182. Dainichi T, Koga H, Tsuji T, Ishii N, Ohyama B, Ueda A, Natsuaki Y, Karashima T, Nakama T, Yasumoto S, Zillikens D, Hashimoto T (2010) From anti-p200 pemphigoid to anti-laminin gamma1 pemphigoid. J Dermatol 37:231–238

    Article  CAS  PubMed  Google Scholar 

  183. Goletz S, Hashimoto T, Zillikens D, Schmidt E (2014) Anti-p200 pemphigoid. J Am Acad Dermatol 71:185–191. doi:10.1016/j.jaad.2014.02.036

    Article  CAS  PubMed  Google Scholar 

  184. Schmidt E, Groves R (2016) Immunobullous diseases. In: Griffith C, Barker J, Chalmers, Bleiker T, Creamer D (Eds) Rook's Textbook of Dermatology, part 3, chapter 50, 9th edition Wiley. Chichester 50:1–56

    Google Scholar 

  185. Geller S, Gat A, Zeeli T, Schmidt E, Ishii N, Hashimoto T, Zillikens D, Sprecher E (2016) A refractory, cutaneous, subepidermal bullous disease. Clin Exp Dermatol 41:573–575. doi:10.1111/ced.12823

    Article  CAS  PubMed  Google Scholar 

  186. Mascaro JM Jr, Zillikens D, Giudice GJ, Caux F, Fleming MG, Katz HM, Diaz LA (2000) A subepidermal bullous eruption associated with IgG autoantibodies to a 200 kd dermal antigen: the first case report from the United States. J Am Acad Dermatol 42:309–315

    Article  PubMed  Google Scholar 

  187. Commin MH, Schmidt E, Duvert-Lehembre S, Lasek A, Morice C, Estival JL, Debarbieux S, Rigal E, Pauwels C, De Quatrebarbes J, Roussel A, Goujon E, Stoebner PE, Jouen F, Joly P (2016) Clinical and immunological features and outcome of anti-p200 pemphigoid. Br J Dermatol 175:776–781. doi:10.1111/bjd.14629

    Article  CAS  PubMed  Google Scholar 

  188. Meijer JM, Diercks GF, Schmidt E, Pas HH, Jonkman MF (2016) Laboratory diagnosis and clinical profile of anti-p200 pemphigoid. JAMA Dermatol 152:897–904. doi:10.1001/jamadermatol.2016.1099

    Article  PubMed  Google Scholar 

  189. Monshi B, Groth S, Richter L, Schmidt E, Zillikens D, Rappersberger K (2012) A long-term study of a patient with anti-p200 pemphigoid: correlation of autoantibody levels with disease activity and an example of epitope spreading. Br J Dermatol 167:1179–1183. doi:10.1111/j.1365-2133.2012.11076.x

    Article  CAS  PubMed  Google Scholar 

  190. Dilling A, Rose C, Hashimoto T, Zillikens D, Shimanovich I (2007) Anti-p200 pemphigoid: a novel autoimmune subepidermal blistering disease. J Dermatol 34:1–8

    Article  PubMed  Google Scholar 

  191. Yamane N, Sawamura D, Nishie W, Abe M, Kodama K, Adachi K, Nakamura H, Ishii N, Hashimoto T, Shimizu H (2007) Anti-p200 pemphigoid in a 17-year-old girl successfully treated with systemic corticosteroid and dapsone. Br J Dermatol 156:1075–1078. doi:10.1111/j.1365-2133.2007.07810.x

    Article  CAS  PubMed  Google Scholar 

  192. Kasperkiewicz M, Hoppe U, Zillikens D, Schmidt E (2010) Relapse-associated autoantibodies to BP180 in a patient with anti-p200 pemphigoid. Clin Exp Dermatol 35:614–617. doi:10.1111/j.1365-2230.2009.03731.x

    Article  CAS  PubMed  Google Scholar 

  193. Shimizu A, Funakoshi T, Ishibashi M, Yoshida T, Koga H, Hashimoto T, Amagai M, Ishiko A (2013) Immunoglobulin G deposition to nonhemidesmosomal lamina lucida and early neutrophil involvement are characteristic features in a case of anti-p200 pemphigoid. Br J Dermatol 168:647–655. doi:10.1111/bjd.12033

    Article  CAS  PubMed  Google Scholar 

  194. Zillikens D, Ishiko A, Jonkman MF, Chimanovitch I, Shimizu H, Hashimoto T, Brocker EB (2000) Autoantibodies in anti-p200 pemphigoid stain skin lacking laminin 5 and type VII collagen. Br J Dermatol 143:1043–1049

    Article  CAS  PubMed  Google Scholar 

  195. Egan CA, Yee C, Zillikens D, Yancey KB (2002) Anti-p200 pemphigoid: diagnosis and treatment of a case presenting as an inflammatory subepidermal blistering disease. J Am Acad Dermatol 46:786–789

    Article  PubMed  Google Scholar 

  196. Hofmann SC, Voith U, Sasaki T, Trueb RM, Nischt R, Bruckner-Tuderman L (2008) The autoantigen in anti-p200 pemphigoid is synthesized by keratinocytes and fibroblasts and is distinct from nidogen-2. J Invest Dermatol 128:87–95. doi:10.1038/sj.jid.5700952

    Article  CAS  PubMed  Google Scholar 

  197. Vafia K, Groth S, Beckmann T, Hirose M, Dworschak J, Recke A, Ludwig RJ, Hashimoto T, Zillikens D, Schmidt E (2012) Pathogenicity of autoantibodies in anti-p200 pemphigoid. PLoS One 7:e41769. doi:10.1371/journal.pone.0041769

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  198. Wozniak K, Hashimoto T, Fukuda S, Ohyama B, Ishii N, Koga H, Dainichi T, Kowalewski C (2011) IgA anti-p200 pemphigoid. Arch Dermatol 147:1306-1310. 10.1001/archdermatol.2011.303

  199. Goto-Ohguchi Y, Nishie W, Akiyama M, Tateishi Y, Aoyagi S, Tsuji-Abe Y, Sawamura D, Ishii N, Hashimoto T, Shimizu H (2009) A severe and refractory case of anti-p200 pemphigoid resulting in multiple skin ulcers and scar formation. Dermatology 218:265–271. doi:10.1159/000182268

    Article  CAS  PubMed  Google Scholar 

  200. Mitsuya J, Hara H, Ito K, Ishii N, Hashimoto T, Terui T (2008) Metastatic ovarian carcinoma-associated subepidermal blistering disease with autoantibodies to both the p200 dermal antigen and the gamma 2 subunit of laminin 5 showing unusual clinical features. Br J Dermatol 158:1354–1357. doi:10.1111/j.1365-2133.2008.08483.x

    Article  CAS  PubMed  Google Scholar 

  201. Yamada T, Suzuki M, Koike Y, Kida K, Murata S, Ishii N, Hashimoto T, Ohtsuki M (2006) A case of epidermolysis bullosa acquisita with autoantibody to anti-p200 pemphigoid antigen and exfoliative esophagitis. Dermatology 212:381–384. doi:10.1159/000092292

    Article  PubMed  Google Scholar 

  202. Pastar Z, Rados J, Lipozencic J, Dobric I, Marinovic B, Ishii N, Hashimoto T (2007) Case of concurrent epidermolysis bullosa acquisita and anti-p200 pemphigoid—how to treat it? Int J Dermatol 46:295–298. doi:10.1111/j.1365-4632.2006.02969.x

  203. Kamata M, Fujita H, Hamanaka T, Takahashi K, Koga H, Hashimoto T, Sato S (2013) Anti-laminin gamma1 pemphigoid accompanied by autoantibodies to laminin alpha3 and gamma2 subunits of laminin-332. JAMA Dermatol 149:1437–1439. doi:10.1001/jamadermatol.2013.5358

    Article  PubMed  Google Scholar 

  204. Li X, Qian H, Ishii N, Yamaya M, Fukuda H, Mukai H, Hirako Y, Hashimoto T (2014) A case of concurrent antilaminin gamma1 pemphigoid and antilaminin-332-type mucous membrane pemphigoid. Br J Dermatol 171:1257–1259. doi:10.1111/bjd.13107

    Article  CAS  PubMed  Google Scholar 

  205. Colognato H, Yurchenco PD (2000) Form and function: the laminin family of heterotrimers. Dev Dyn 218:213-234. 10.1002/(SICI)1097-0177(200006)218:2<213::AID-DVDY1>3.0.CO;2-R

  206. Rose C, Weyers W, Denisjuk N, Hillen U, Zillikens D, Shimanovich I (2007) Histopathology of anti-p200 pemphigoid. Am J Dermatopathol 29:119-124. 10.1097/DAD.0b013e31803326e6

  207. Koga H, Ishii N, Dainichi T, Tsuruta D, Hamada T, Ohata C, Karashima T, Furumura M, Hashimoto T (2013) An attempt to develop mouse model for anti-laminin gamma1 pemphigoid. J Dermatol Sci 70:108-115. 10.1016/j.jdermsci.2013.01.001

  208. Florea F, Bernards C, Caproni M, Kleindienst J, Hashimoto T, Koch M, Sitaru C (2014) Ex vivo pathogenicity of anti-laminin gamma1 autoantibodies. Am J Pathol 184:494–506. doi:10.1016/j.ajpath.2013.10.019

    Article  CAS  PubMed  Google Scholar 

  209. Iwata H, Hiramitsu Y, Aoyama Y, Kitajima Y (2009) A case of anti-p200 pemphigoid: evidence for a different pathway in neutrophil recruitment compared with bullous pemphigoid. Br J Dermatol 160:462–464. doi:10.1111/j.1365-2133.2008.08965.x

    Article  CAS  PubMed  Google Scholar 

  210. Majima Y, Yagi H, Tateishi C, Groth S, Schmidt E, Zillikens D, Koga H, Hashimoto T, Tokura Y (2013) A successful treatment with ustekinumab in a case of antilaminin-gamma1 pemphigoid associated with psoriasis. Br J Dermatol 168:1367–1369. doi:10.1111/bjd.12163

    Article  CAS  PubMed  Google Scholar 

  211. Kasperkiewicz M, Hoppe U, Zillikens D, Schmidt E (2009) Relapse-associated autoantibodies to BP180 in a patient with anti-p200 pemphigoid. Clin Exp Dermatol in press

  212. Alloo A, Strazzula L, Rothschild B, Hawryluk E, Levine D, Hoang MP, Koga H, Hashimoto T, Kroshinsky D (2014) Refractory antilaminin gamma1 pemphigoid successfully treated with intravenous immunoglobulin and mycophenolate mofetil. J Eur Acad Dermatol Venereol 28:1401–1403. doi:10.1111/jdv.12352

    Article  CAS  PubMed  Google Scholar 

  213. Ohata C, Fukuda S, Ishii N, Koga H, Hamada T, Furumura M, Hashimoto T (2013) Refractory anti-laminin gamma1 pemphigoid with psoriasis vulgaris successfully treated by double-filtration plasmapheresis. Eur J Dermatol 23:715–716. doi:10.1684/ejd.2013.2138

    PubMed  Google Scholar 

  214. Woodley DT, Briggaman RA, O'Keefe EJ, Inman AO, Queen LL, Gammon WR (1984) Identification of the skin basement-membrane autoantigen in epidermolysis bullosa acquisita. N Engl J Med 310:1007–1013

    Article  CAS  PubMed  Google Scholar 

  215. Bernard P, Vaillant L, Labeille B, Bedane C, Arbeille B, Denoeux JP, Lorette G, Bonnetblanc JM, Prost C (1995) Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group. Arch Dermatol 131:48–52

    Article  CAS  PubMed  Google Scholar 

  216. Hubner F, Recke A, Zillikens D, Linder R, Schmidt E (2016) Prevalence and age distribution of pemphigus and pemphigoid diseases in Germany. J Invest Dermatol. doi:10.1016/j.jid.2016.07.013

  217. Gammon WR, Heise ER, Burke WA, Fine JD, Woodley DT, Briggaman RA (1988) Increased frequency of HLA-DR2 in patients with autoantibodies to epidermolysis bullosa acquisita antigen: evidence that the expression of autoimmunity to type VII collagen is HLA class II allele associated. J Invest Dermatol 91:228–232

    Article  CAS  PubMed  Google Scholar 

  218. Zumelzu C, Le Roux-Villet C, Loiseau P, Busson M, Heller M, Aucouturier F, Pendaries V, Lievre N, Pascal F, Brette MD, Doan S, Charron D, Caux F, Laroche L, Petit A, Prost-Squarcioni C (2011) Black patients of African descent and HLA-DRB1*15:03 frequency overrepresented in epidermolysis bullosa acquisita. J Invest Dermatol 131:2386–2393. jid2011231 [pii]. doi:10.1038/jid.2011.231

    Article  CAS  PubMed  Google Scholar 

  219. Zhao CY, Murrell DF Autoimmune blistering diseases in females: a review. International Journal of Women's Dermatology 1:4–12. 10.1016/j.ijwd.2015.01.002

  220. Vorobyev A, Ludwig RJ, Schmidt E (2017) Clinical features and diagnosis of epidermolysis bullosa acquisita. Expert Rev Clin Immunol 13:157–169. doi:10.1080/1744666x.2016.1221343

    Article  CAS  PubMed  Google Scholar 

  221. Schmidt E, Zillikens D (2011) The diagnosis and treatment of autoimmune blistering skin diseases. Dtsch Arztebl Int 108:399-405, i-iii. 10.3238/arztebl.2011.0405

  222. Prost Squarcioni C, Caux F (2015). Epidermolysis Bullosa Berlin Heidelberg

  223. Sebaratnam DF, Frew JW, Davatchi F, Murrell DF (2012) Quality-of-life measurement in blistering diseases. Dermatol Clin 30(301–307):ix. doi:10.1016/j.det.2011.11.008

    Google Scholar 

  224. Terra JB, Meijer JM, Jonkman MF, Diercks GF (2013) The n- vs. u-serration is a learnable criterion to differentiate pemphigoid from epidermolysis bullosa acquisita in direct immunofluorescence serration pattern analysis. Br J Dermatol 169:100–105. doi:10.1111/bjd.12308

    Article  CAS  PubMed  Google Scholar 

  225. Vodegel RM, Jonkman MF, Pas HH, de Jong MC (2004) U-serrated immunodeposition pattern differentiates type VII collagen targeting bullous diseases from other subepidermal bullous autoimmune diseases. Br J Dermatol 151:112–118. doi:10.1111/j.1365-2133.2004.06006.x

    Article  CAS  PubMed  Google Scholar 

  226. Komorowski L, Muller R, Vorobyev A, Probst C, Recke A, Jonkman MF, Hashimoto T, Kim SC, Groves R, Ludwig RJ, Zillikens D, Stocker W, Schmidt E (2013) Sensitive and specific assays for routine serological diagnosis of epidermolysis bullosa acquisita. J Am Acad Dermatol 68:e89–e95. doi:10.1016/j.jaad.2011.12.032

    Article  CAS  PubMed  Google Scholar 

  227. Saleh MA, Ishii K, Kim YJ, Murakami A, Ishii N, Hashimoto T, Schmidt E, Zillikens D, Shirakata Y, Hashimoto K, Kitajima Y, Amagai M (2011) Development of NC1 and NC2 domains of type VII collagen ELISA for the diagnosis and analysis of the time course of epidermolysis bullosa acquisita patients. J Dermatol Sci 62:169–175. doi:10.1016/j.jdermsci.2011.03.003

    Article  CAS  PubMed  Google Scholar 

  228. van Beek N, Dahnrich C, Johannsen N et al (2016) Prospective studies on the routine use of a novel multivariant enzyme-linked immunosorbent assay for the diagnosis of autoimmune bullous diseases. J Am Acad Dermatol. doi:10.1016/j.jaad.2016.11.002

  229. Calabresi V, Sinistro A, Cozzani E, Cerasaro C, Lolicato F, Muscianese M, Parodi A, Didona B, Zambruno G, Di Zenzo G (2014) Sensitivity of different assays for the serological diagnosis of epidermolysis bullosa acquisita: analysis of a cohort of 24 Italian patients. J Eur Acad Dermatol Venereol 28:483–490. doi:10.1111/jdv.12129

    Article  CAS  PubMed  Google Scholar 

  230. Seta V, Aucouturier F, Bonnefoy J, Le Roux-Villet C, Pendaries V, Alexandre M, Grootenboer-Mignot S, Heller M, Lievre N, Laroche L, Caux F, Titeux M, Hovnanian A, Prost-Squarcioni C (2016) Comparison of 3 type VII collagen (C7) assays for serologic diagnosis of epidermolysis bullosa acquisita (EBA). J Am Acad Dermatol 74:1166–1172. doi:10.1016/j.jaad.2016.01.005

    Article  CAS  PubMed  Google Scholar 

  231. Chen M, Chan LS, Cai X, O'Toole EA, Sample JC, Woodley DT (1997) Development of an ELISA for rapid detection of anti-type VII collagen autoantibodies in epidermolysis bullosa acquisita. J Invest Dermatol 108:68–72

    Article  CAS  PubMed  Google Scholar 

  232. De Jong MC, Bruins S, Heeres K, Jonkman MF, Nieboer C, Boorsma DM, Pas HH, van der Meer JB (1996) Bullous pemphigoid and epidermolysis bullosa acquisita. Differentiation by fluorescence overlay antigen mapping. Arch Dermatol 132:151–157

    Article  PubMed  Google Scholar 

  233. Yaoita H, Briggaman RA, Lawley TJ, Provost TT, Katz SI (1981) Epidermolysis bullosa acquisita: ultrastructural and immunological studies. J Invest Dermatol 76:288–292

    Article  CAS  PubMed  Google Scholar 

  234. Prost C, Dubertret L, Fosse M, Wechsler J, Touraine R (1984) A routine immuno-electron microscopic technique for localizing an auto-antibody on epidermal basement membrane. Br J Dermatol 110:1–7

    Article  CAS  PubMed  Google Scholar 

  235. Prost Squarcioni C (2015). Electron microscopy and immunoelectron microscopy. Berlin Heidelberg

    Book  Google Scholar 

  236. Ludwig RJ (2013) Clinical presentation, pathogenesis, diagnosis, and treatment of epidermolysis bullosa acquisita. ISRN Dermatol 2013:812029. doi:10.1155/2013/812029

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  237. Kasperkiewicz M, Sadik CD, Bieber K, Ibrahim SM, Manz RA, Schmidt E, Zillikens D, Ludwig RJ (2016) Epidermolysis bullosa acquisita: from pathophysiology to novel therapeutic options. J Invest Dermatol 136:24–33. doi:10.1038/JID.2015.356

    Article  CAS  PubMed  Google Scholar 

  238. Kasperkiewicz M, Zillikens D, Schmidt E (2011) Pemphigoid diseases: pathogenesis, diagnosis, and treatment. Autoimmunity 45:55–70. doi:10.3109/08916934.2011.606447

    Article  PubMed  CAS  Google Scholar 

  239. Prost-Squarcioni C, Caux F (2015) Epidermolysis Bullosa. Berlin, Heidelberg

    Google Scholar 

  240. Kirtschig G, Murrell D, Wojnarowska F, Khumalo N (2003) Interventions for mucous membrane pemphigoid and epidermolysis bullosa acquisita. Cochrane Database Syst Rev:Cd004056. 10.1002/14651858.cd004056

  241. Intong LR, Murrell DF (2011) Management of epidermolysis bullosa acquisita. Dermatol Clin 29:643–647. doi:10.1016/j.det.2011.06.020

    Article  CAS  PubMed  Google Scholar 

  242. Gupta R, Woodley DT, Chen M (2012) Epidermolysis bullosa acquisita. Clin Dermatol 30:60–69. doi:10.1016/j.clindermatol.2011.03.011

    Article  PubMed Central  PubMed  Google Scholar 

  243. Schmidt E, Benoit S, Brocker EB, Zillikens D, Goebeler M (2006) Successful adjuvant treatment of recalcitrant epidermolysis bullosa acquisita with anti-CD20 antibody rituximab. Arch Dermatol 142:147–150

    Article  PubMed  Google Scholar 

  244. Schmidt E, Brocker EB, Goebeler M (2008) Rituximab in treatment-resistant autoimmune blistering skin disorders. Clin Rev Allergy Immunol 34:56–64

    Article  CAS  PubMed  Google Scholar 

  245. Daniel BS, Murrell DF, Joly P (2011) Rituximab and its use in autoimmune bullous disorders. Dermatol Clin 29:571–575. doi:10.1016/j.det.2011.06.023

    Article  CAS  PubMed  Google Scholar 

  246. Chee SN, Murrell DF (2011) The use of intravenous immunoglobulin in autoimmune bullous diseases. Dermatol Clin 29:565–570. doi:10.1016/j.det.2011.06.010

    Article  CAS  PubMed  Google Scholar 

  247. Schmidt E, Zillikens D (2010) Immunoadsorption in dermatology. Arch Dermatol Res 302:241–253

    Article  PubMed  Google Scholar 

  248. Brennan F, Lim C (2015). The pathogenesis, assessment and management of pruritus in autoimmune bullous diseases;. Berlin Heidelberg

    Book  Google Scholar 

  249. Zillikens D, Wever S, Roth A, Weidenthaler-Barth B, Hashimoto T, Brocker EB (1995) Incidence of autoimmune subepidermal blistering dermatoses in a region of central Germany. Arch Dermatol 131:957–958

    Article  CAS  PubMed  Google Scholar 

  250. Radford CF, Rauz S, Williams GP, Saw VP, Dart JK (2012) Incidence, presenting features, and diagnosis of cicatrising conjunctivitis in the United Kingdom. Eye (Lond) 26:1199–1208. doi:10.1038/eye.2012.119

    Article  CAS  Google Scholar 

  251. Chan LS, Hammerberg C, Cooper KD (1997) Significantly increased occurrence of HLA-DQB1*0301 allele in patients with ocular cicatricial pemphigoid. J Invest Dermatol 108:129–132

    Article  CAS  PubMed  Google Scholar 

  252. Carrozzo M, Fasano ME, Broccoletti R, Carbone M, Cozzani E, Rendine S, Roggero S, Parodi A, Gandolfo S (2001) HLA-DQB1 alleles in Italian patients with mucous membrane pemphigoid predominantly affecting the oral cavity. Br J Dermatol 145:805–808

    Article  CAS  PubMed  Google Scholar 

  253. Chan LS, Ahmed AR, Anhalt GJ et al (2002) The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol 138:370–379

    PubMed  Google Scholar 

  254. Murrell DF, Marinovic B, Caux F et al (2015) Definitions and outcome measures for mucous membrane pemphigoid: recommendations of an international panel of experts. J Am Acad Dermatol 72:168–174. doi:10.1016/j.jaad.2014.08.024

    Article  PubMed  Google Scholar 

  255. Egan CA, Lazarova Z, Darling TN, Yee C, Cote T, Yancey KB (2001) Anti-epiligrin cicatricial pemphigoid and relative risk for cancer. Lancet 357:1850–1851. doi:10.1016/s0140-6736(00)04971-0

    Article  CAS  PubMed  Google Scholar 

  256. Matsushima S, Horiguchi Y, Honda T, Fujii S, Okano T, Tanabe M, Wakayama T, Hashimoto T, Yancey KB (2004) A case of anti-epiligrin cicatricial pemphigoid associated with lung carcinoma and severe laryngeal stenosis: review of Japanese cases and evaluation of risk for internal malignancy. J Dermatol 31:10–15

    Article  PubMed  Google Scholar 

  257. Sebaratnam DF, Hanna AM, Chee SN, Frew JW, Venugopal SS, Daniel BS, Martin LK, Rhodes LM, Tan JC, Wang CQ, Welsh B, Nijsten T, Murrell DF (2013) Development of a quality-of-life instrument for autoimmune bullous disease: the autoimmune bullous disease quality of life questionnaire. JAMA Dermatol 149:1186–1191. doi:10.1001/jamadermatol.2013.4972

    Article  PubMed  Google Scholar 

  258. Tjokrowidjaja A, Daniel BS, Frew JW, Sebaratnam DF, Hanna AM, Chee S, Dermawan A, Wang CQ, Lim C, Venugopal SS, Rhodes LM, Welsh B, Nijsten T, Murrell DF (2013) The development and validation of the treatment of autoimmune bullous disease quality of life questionnaire, a tool to measure the quality of life impacts of treatments used in patients with autoimmune blistering disease. Br J Dermatol 169:1000–1006. doi:10.1111/bjd.12623

    Article  CAS  PubMed  Google Scholar 

  259. Megahed M, Schmiedeberg S, Becker J, Ruzicka T (2001) Treatment of cicatricial pemphigoid with mycophenolate mofetil as a steroid-sparing agent. J Am Acad Dermatol 45:256–259. doi:10.1067/mjd.2001.114746

    Article  CAS  PubMed  Google Scholar 

  260. Ingen-Housz-Oro S, Prost-Squarcioni C, Pascal F, Doan S, Brette MD, Bachelez H, Dubertret L (2005) Cicatricial pemphigoid: treatment with mycophenolate mofetil. Ann Dermatol Venereol 132:13–16

    Article  CAS  PubMed  Google Scholar 

  261. Doycheva D, Deuter C, Blumenstock G, Stuebiger N, Zierhut M (2011) Long-term results of therapy with mycophenolate mofetil in ocular mucous membrane pemphigoid. Ocul Immunol Inflamm 19:431–438. doi:10.3109/09273948.2011.624288

    Article  CAS  PubMed  Google Scholar 

  262. Munyangango EM, Le Roux-Villet C, Doan S, Pascal F, Soued I, Alexandre M, Heller M, Lievre N, Aucouturier F, Caux F, Laroche L, Prost-Squarcioni C (2013) Oral cyclophosphamide without corticosteroids to treat mucous membrane pemphigoid. Br J Dermatol 168:381–390. doi:10.1111/bjd.12041

    Article  CAS  PubMed  Google Scholar 

  263. Musette P, Pascal F, Hoang-Xuan T, Heller M, Lok C, Deboise A, Dubertret L, Prost C (2001) Treatment of cicatricial pemphigoid with pulse intravenous cyclophosphamide. Arch Dermatol 137:101–102

    CAS  PubMed  Google Scholar 

  264. Foster CS, Chang PY, Ahmed AR (2010) Combination of rituximab and intravenous immunoglobulin for recalcitrant ocular cicatricial pemphigoid: a preliminary report. Ophthalmology 117:861–869. doi:10.1016/j.ophtha.2009.09.049

    Article  PubMed  Google Scholar 

  265. Le Roux-Villet C, Prost-Squarcioni C, Alexandre M, Caux F, Pascal F, Doan S, Brette MD, Soued I, Gabison E, Aucouturier F, Letestu R, Laroche L, Bachelez H (2011) Rituximab for patients with refractory mucous membrane pemphigoid. Arch Dermatol 147:843–849. doi:10.1001/archdermatol.2011.54

    Article  PubMed  CAS  Google Scholar 

  266. Lourari S, Herve C, Doffoel-Hantz V, Meyer N, Bulai-Livideanu C, Viraben R, Maza A, Adoue D, Bedane C, Paul C (2011) Bullous and mucous membrane pemphigoid show a mixed response to rituximab: experience in seven patients. J Eur Acad Dermatol Venereol 25:1238–1240. doi:10.1111/j.1468-3083.2010.03889.x

    Article  CAS  PubMed  Google Scholar 

  267. Sladden C, Kirchhof MG, Crawford RI (2014) Biopsy location for direct immunofluorescence in patients with suspected bullous pemphigoid impacts probability of a positive test result. J Cutan Med Surg 18:392–396. doi:10.2310/7750.2014.14004

    Article  PubMed  Google Scholar 

  268. Schmidt E, Goebeler M, Hertl M, Sardy M, Sitaru C, Eming R, Hofmann SC, Hunzelmann N, Kern JS, Kramer H, Orzechowski HD, Pfeiffer C, Schuster V, Sporbeck B, Sticherling M, Worm M, Zillikens D, Nast A (2015) S2k guideline for the diagnosis of pemphigus vulgaris/foliaceus and bullous pemphigoid. J Dtsch Dermatol Ges 13:713–727. doi:10.1111/ddg.12612

    PubMed  Google Scholar 

  269. Glauser S, Rutz M, Cazzaniga S, Hegyi I, Borradori L, Beltraminelli H (2016) Diagnostic value of immunohistochemistry on formalin-fixed, paraffin-embedded skin biopsy specimens for bullous pemphigoid. Br J Dermatol 175:988–993. doi:10.1111/bjd.14686

    Article  CAS  PubMed  Google Scholar 

  270. Sardy M, Kostaki D, Varga R, Peris K, Ruzicka T (2013) Comparative study of direct and indirect immunofluorescence and of bullous pemphigoid 180 and 230 enzyme-linked immunosorbent assays for diagnosis of bullous pemphigoid. J Am Acad Dermatol 69:748–753. doi:10.1016/j.jaad.2013.07.009

    Article  CAS  PubMed  Google Scholar 

  271. Buch AC, Kumar H, Panicker N, Misal S, Sharma Y, Gore CR (2014) A cross-sectional study of direct immunofluorescence in the diagnosis of immunobullous dermatoses. Indian J Dermatol 59:364–368. doi:10.4103/0019-5154.135488

    Article  PubMed Central  PubMed  Google Scholar 

  272. Chaidemenos GC, Maltezos E, Chrysomallis F, Kouskoukis K, Kapetis E, Mourellou O, Gotsis N (1998) Value of routine diagnostic criteria of bullous pemphigoid. Int J Dermatol 37:206–210

    Article  CAS  PubMed  Google Scholar 

  273. Mysorekar VV, Sumathy TK, Shyam Prasad AL (2015) Role of direct immunofluorescence in dermatological disorders. Indian Dermatol Online J 6:172–180. doi:10.4103/2229-5178.156386

    Article  PubMed Central  PubMed  Google Scholar 

  274. Vaillant L, Bernard P, Joly P, Prost C, Labeille B, Bedane C, Arbeille B, Thomine E, Bertrand P, Lok C, Roujeau JC (1998) Evaluation of clinical criteria for diagnosis of bullous pemphigoid. French Bullous Study Group. Arch Dermatol 134:1075–1080

    Article  CAS  PubMed  Google Scholar 

  275. Pohla-Gubo G, Hintner H (2011) Direct and indirect immunofluorescence for the diagnosis of bullous autoimmune diseases. Dermatol Clin 29(365–372):vii. doi:10.1016/j.det.2011.03.001

    Google Scholar 

  276. Kelly SE, Wojnarowska F (1988) The use of chemically split tissue in the detection of circulating anti-basement membrane zone antibodies in bullous pemphigoid and cicatricial pemphigoid. Br J Dermatol 118:31–40

    Article  CAS  PubMed  Google Scholar 

  277. Ghohestani RF, Nicolas JF, Rousselle P, Claudy AL (1997) Diagnostic value of indirect immunofluorescence on sodium chloride-split skin in differential diagnosis of subepidermal autoimmune bullous dermatoses. Arch Dermatol 133:1102–1107

    Article  CAS  PubMed  Google Scholar 

  278. Yang B, Wang C, Chen S, Chen X, Zhou G, Tian H, Yu M, Zhang D, Shi Z, Zhang F (2011) Accuracy of indirect immunofluorescence on sodium chloride-split skin in the differential diagnosis of bullous pemphigoid and epidermolysis bullosa acquisita. Indian J Dermatol Venereol Leprol 77:677–682. doi:10.4103/0378-6323.86479

    Article  PubMed  Google Scholar 

  279. Otten JV, Hashimoto T, Hertl M, Payne AS, Sitaru C (2014) Molecular diagnosis in autoimmune skin blistering conditions. Curr Mol Med 14:69–95

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  280. Sitaru C, Dahnrich C, Probst C, Komorowski L, Blocker I, Schmidt E, Schlumberger W, Rose C, Stocker W, Zillikens D (2007) Enzyme-linked immunosorbent assay using multimers of the 16th non-collagenous domain of the BP180 antigen for sensitive and specific detection of pemphigoid autoantibodies. Exp Dermatol 16:770–777. doi:10.1111/j.1600-0625.2007.00592.x

    Article  CAS  PubMed  Google Scholar 

  281. Blocker IM, Dahnrich C, Probst C, Komorowski L, Saschenbrecker S, Schlumberger W, Stocker W, Zillikens D, Schmidt E (2012) Epitope mapping of BP230 leading to a novel enzyme-linked immunosorbent assay for autoantibodies in bullous pemphigoid. Br J Dermatol 166:964–970. doi:10.1111/j.1365-2133.2012.10820.x

    Article  CAS  PubMed  Google Scholar 

  282. Sitaru C, Powell J, Messer G, Brocker EB, Wojnarowska F, Zillikens D (2004) Immunoblotting and enzyme-linked immunosorbent assay for the diagnosis of pemphigoid gestationis. Obstet Gynecol 103:757–763. doi:10.1097/01.AOG.0000115506.76104.ad

    Article  PubMed  Google Scholar 

  283. Sadik CD, Lima AL, Zillikens D (2016) Pemphigoid gestationis: toward a better understanding of the etiopathogenesis. Clin Dermatol 34:378–382. doi:10.1016/j.clindermatol.2016.02.010

    Article  PubMed  Google Scholar 

  284. Schmidt E, Obe K, Brocker EB, Zillikens D (2000) Serum levels of autoantibodies to BP180 correlate with disease activity in patients with bullous pemphigoid. Arch Dermatol 136:174–178

    Article  CAS  PubMed  Google Scholar 

  285. Charneux J, Lorin J, Vitry F, Antonicelli F, Reguiai Z, Barbe C, Tabary T, Grange F, Bernard P (2011) Usefulness of BP230 and BP180-NC16a enzyme-linked immunosorbent assays in the initial diagnosis of bullous pemphigoid: a retrospective study of 138 patients. Arch Dermatol 147:286–291. doi:10.1001/archdermatol.2011.23

    Article  PubMed  Google Scholar 

  286. Di Zenzo G, Thoma-Uszynski S, Fontao L, Calabresi V, Hofmann SC, Hellmark T, Sebbag N, Pedicelli C, Sera F, Lacour JP, Wieslander J, Bruckner-Tuderman L, Borradori L, Zambruno G, Hertl M (2008) Multicenter prospective study of the humoral autoimmune response in bullous pemphigoid. Clin Immunol 128:415–426. doi:10.1016/j.clim.2008.04.012

    Article  PubMed  CAS  Google Scholar 

  287. Kromminga A, Sitaru C, Hagel C, Herzog S, Zillikens D (2004) Development of an ELISA for the detection of autoantibodies to BP230. Clin Immunol 111:146–152. doi:10.1016/j.clim.2003.12.007

    Article  CAS  PubMed  Google Scholar 

  288. Le Sache-de Peufeilhoux L, Ingen-Housz-Oro S, Hue S, Sbidian E, Valeyrie-Allanore L, Ortonne N, Roujeau JC, Wolkenstein P, Chosidow O, Andre C (2012) The value of BP230 enzyme-linked immunosorbent assay in the diagnosis and immunological follow-up of bullous pemphigoid. Dermatology 224:154–159. doi:10.1159/000337545

    Article  PubMed  CAS  Google Scholar 

  289. Keller JJ, Kittridge AL, Debanne SM, Korman NJ (2016) Evaluation of ELISA testing for BP180 and BP230 as a diagnostic modality for bullous pemphigoid: a clinical experience. Arch Dermatol Res 308:269–272. doi:10.1007/s00403-016-1631-1

    Article  CAS  PubMed  Google Scholar 

  290. Ingen-Housz-Oro S, Plee J, Belmondo T, Maizieres M, Pham BN, Hue S, Ortonne N, Durlach A, Wolkenstein P, Chosidow O, Bernard P (2015) Positive direct immunofluorescence is of better value than ELISA-BP180 and ELISA-BP230 values for the prediction of relapse after treatment cessation in bullous pemphigoid: a retrospective study of 97 patients. Dermatology 231:50–55. doi:10.1159/000381143

    Article  CAS  PubMed  Google Scholar 

  291. Yang B, Wang C, Chen S, Chen X, Lu X, Tian H, Yu M, Zhang D, Shi Z, Zhou G, Zhang F (2012) Evaluation of the combination of BP180-NC16a enzyme-linked immunosorbent assay and BP230 enzyme-linked immunosorbent assay in the diagnosis of bullous pemphigoid. Indian J Dermatol Venereol Leprol 78:722–727. doi:10.4103/0378-6323.102364

    Article  PubMed  Google Scholar 

  292. Lee EH, Kim YH, Kim S, Kim SE, Kim SC (2012) Usefulness of enzyme-linked immunosorbent assay using recombinant BP180 and BP230 for serodiagnosis and monitoring disease activity of bullous pemphigoid. Ann Dermatol 24:45–55. doi:10.5021/ad.2012.24.1.45

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  293. Tampoia M, Giavarina D, Di Giorgio C, Bizzaro N (2012) Diagnostic accuracy of enzyme-linked immunosorbent assays (ELISA) to detect anti-skin autoantibodies in autoimmune blistering skin diseases: a systematic review and meta-analysis. Autoimmun Rev 12:121–126. doi:10.1016/j.autrev.2012.07.006

    Article  CAS  PubMed  Google Scholar 

  294. van Beek N, Rentzsch K, Probst C, Komorowski L, Kasperkiewicz M, Fechner K, Bloecker IM, Zillikens D, Stocker W, Schmidt E (2012) Serological diagnosis of autoimmune bullous skin diseases: prospective comparison of the BIOCHIP mosaic-based indirect immunofluorescence technique with the conventional multi-step single test strategy. Orphanet J Rare Dis 7:49. doi:10.1186/1750-1172-7-49

    Article  PubMed Central  PubMed  Google Scholar 

  295. Tampoia M, Zucano A, Villalta D, Antico A, Bizzaro N (2012) Anti-skin specific autoantibodies detected by a new immunofluorescence multiplex biochip method in patients with autoimmune bullous diseases. Dermatology 225:37–44. doi:10.1159/000339776

    Article  CAS  PubMed  Google Scholar 

  296. Russo I, Saponeri A, Peserico A, Alaibac M (2014) The use of biochip immunofluorescence microscopy for the diagnosis of pemphigus vulgaris. Acta Histochem 116:713–716. doi:10.1016/j.acthis.2013.12.012

    Article  CAS  PubMed  Google Scholar 

  297. Gornowicz-Porowska J, Dmochowski M, Pietkiewicz P, Bowszyc-Dmochowska M (2015) Mucosal-dominant pemphigus vulgaris in a captopril-taking woman with angioedema. An Bras Dermatol 90:748–751. doi:10.1590/abd1806-4841.20153390

    Article  PubMed Central  PubMed  Google Scholar 

  298. van Beek N, Dohse A, Riechert F, Krull V, Recke A, Zillikens D, Schmidt E (2014) Serum autoantibodies against the dermal-epidermal junction in patients with chronic pruritic disorders, elderly individuals and blood donors prospectively recruited. Br J Dermatol 170:943–947. doi:10.1111/bjd.12739

    Article  PubMed  CAS  Google Scholar 

  299. Kokkonen N, Herukka SK, Huilaja L, Kokki M, Koivisto AM, Hartikainen P, Remes AM, Tasanen K (2017) Increased levels of the bullous pemphigoid BP180 autoantibody are associated with more severe dementia in Alzheimer's disease. J Invest Dermatol 137:71–76. doi:10.1016/j.jid.2016.09.010

    Article  CAS  PubMed  Google Scholar 

  300. Amber KT, Bloom R, Hertl M (2016) A systematic review with pooled analysis of clinical presentation and immunodiagnostic testing in mucous membrane pemphigoid: association of anti-laminin-332 IgG with oropharyngeal involvement and the usefulness of ELISA. J Eur Acad Dermatol Venereol 30:72–77. doi:10.1111/jdv.13397

    Article  CAS  PubMed  Google Scholar 

  301. Bernard P, Antonicelli F, Bedane C, Joly P, Le Roux-Villet C, Duvert-Lehembre S, Rousselle P, Prost-Squarcioni C (2013) Prevalence and clinical significance of anti-laminin 332 autoantibodies detected by a novel enzyme-linked immunosorbent assay in mucous membrane pemphigoid. JAMA Dermatol 149:533–540. doi:10.1001/jamadermatol.2013.1434

    Article  CAS  PubMed  Google Scholar 

  302. Fania L, Caldarola G, Muller R, Brandt O, Pellicano R, Feliciani C, Hertl M (2012) IgE recognition of bullous pemphigoid (BP)180 and BP230 in BP patients and elderly individuals with pruritic dermatoses. Clin Immunol 143:236–245. doi:10.1016/j.clim.2012.02.003

    Article  CAS  PubMed  Google Scholar 

  303. Terra JB, Jonkman MF, Diercks GF, Pas HH (2013) Low sensitivity of type VII collagen enzyme-linked immunosorbent assay in epidermolysis bullosa acquisita: serration pattern analysis on skin biopsy is required for diagnosis. Br J Dermatol 169:164–167. doi:10.1111/bjd.12300

    Article  CAS  PubMed  Google Scholar 

  304. Licarete E, Ganz S, Recknagel MJ, Di Zenzo G, Hashimoto T, Hertl M, Zambruno G, Hundorfean G, Mudter J, Neurath MF, Bruckner-Tuderman L, Sitaru C (2012) Prevalence of collagen VII-specific autoantibodies in patients with autoimmune and inflammatory diseases. BMC Immunol 13:16. doi:10.1186/1471-2172-13-16

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  305. Vodegel RM, de Jong MC, Pas HH, Yancey KB, Jonkman MF (2003) Anti-epiligrin cicatricial pemphigoid and epidermolysis bullosa acquisita: differentiation by use of indirect immunofluorescence microscopy. J Am Acad Dermatol 48:542–547. doi:10.1067/mjd.2003.99

    Article  PubMed  Google Scholar 

  306. Schmidt E, Zillikens D (2010) Modern diagnosis of autoimmune blistering skin diseases. Autoimmun Rev 10:84–89. doi:10.1016/j.autrev.2010.08.007

    Article  CAS  PubMed  Google Scholar 

  307. Marzano AV, Cozzani E, Biasin M, Russo I, Alaibac M (2016) The use of biochip immunofluorescence microscopy for the serological diagnosis of epidermolysis bullosa acquisita. Arch Dermatol Res 308:273–276. doi:10.1007/s00403-016-1632-0

    Article  CAS  PubMed  Google Scholar 

  308. Hayakawa T, Furumura M, Fukano H, Li X, Ishii N, Hamada T, Ohata C, Tsuruta D, Shimozato K, Hashimoto T (2014) Diagnosis of oral mucous membrane pemphigoid by means of combined serologic testing. Oral Surg Oral Med Oral Pathol Oral Radiol 117:483–496. doi:10.1016/j.oooo.2013.12.402

    Article  PubMed  Google Scholar 

  309. Kazama T, Yamamoto Y, Hashimoto T, Komai A, Ito M (1998) Application of confocal laser scanning microscopy to differential diagnosis of bullous pemphigoid and epidermolysis bullosa acquisita. Br J Dermatol 138:593–601

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Dermatology, University of California Irvine Health, 118 Med Surg 1, Irvine, CA, 92697, USA

    Kyle T. Amber

  2. Department of Dermatology, St. George Hospital, Gray Street, Kogarah, Sydney, NSW, Australia

    Dedee F. Murrell

  3. Department of Dermatology, University of Lübeck, Lübeck, Germany

    Enno Schmidt

  4. Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany

    Enno Schmidt

  5. Department of Dermatology, INSERM U901, University of Rouen, Rouen, France

    Pascal Joly

  6. Department of Dermatology, University of Bern, Bern, Switzerland

    Luca Borradori

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  1. Kyle T. Amber
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  2. Dedee F. Murrell
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Amber, K.T., Murrell, D.F., Schmidt, E. et al. Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management. Clinic Rev Allerg Immunol 54, 26–51 (2018). https://doi.org/10.1007/s12016-017-8633-4

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