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Blistering Disorders and Acantholytic Processes Affecting the Epidermis of the Vulva

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Vulvar Pathology

Abstract

Although uncommon, vulvar involvement can be the initial or even the sole manifestation of a blistering or acantholytic condition. Classification of these vesiculobullous conditions is based on the anatomic location of the blister—whether the separation is within the stratum corneum, within the epidermis, or below the epidermis.

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References

  1. Patel NN, Patel DN. Staphylococcal scalded skin syndrome. Am J Med. 2010;123(6):505–7. doi:10.1016/j.amjmed.2009.09.041.

    Article  PubMed  Google Scholar 

  2. Amagai M, Matsuyoshi N, Wang ZH, Andl C, Stanley JR. Toxin is bullous impetigo and staphylococcal scalded-skin syndrome targets desmoglein 1. Nat Med. 2000;6(11):1275–7.

    Article  CAS  PubMed  Google Scholar 

  3. Monday SR, Vath GM, Ferens WA, Deobald C, Rago JV, Gahr PJ, et al. Unique superantigen activity of staphylococcal exfoliative toxins. J Immunol. 1999;162(8):4550–9.

    CAS  PubMed  Google Scholar 

  4. Fischer G, Rogers M. Vulvar disease in children: a clinical audit of 130 cases. Pediatr Dermatol. 2000;17(1):1–6.

    Article  CAS  PubMed  Google Scholar 

  5. Ladhani S. Understanding the mechanism of action of the exfoliative toxins of Staphylococcus aureus. FEMS Immunol Med Microbiol. 2003;39(2):181–9.

    Article  CAS  PubMed  Google Scholar 

  6. Patel GK, Finlay AY. Staphylococcal scalded skin syndrome: diagnosis and management. Am J Clin Dermatol. 2003;4(3):165–75.

    Article  PubMed  Google Scholar 

  7. Amagai M. Pemphigus: autoimmunity to epidermal cell adhesion molecules. Adv Dermatol. 1996;11:319–52.

    CAS  PubMed  Google Scholar 

  8. Barbosa NDF, de Aguiar LM, Maruta CW, Aoki V, Sotto MN, Labinas GH, et al. Vulvo-cervico-vaginal manifestations and evaluation of Papanicolaou smears in pemphigus vulgaris and pemphigus foliaceus. J Am Acad Dermatol. 2012;67(3):409–16.

    Article  Google Scholar 

  9. Qian Y, Jeong JS, Maldonado M, Valenzuela JG, Gomes R, Teixeira C, et al. Cutting edge: Brazilian pemphigus foliaceus anti-desmoglein 1 autoantibodies cross-react with sand fly salivary LJM11 antigen. J Immunol. 2012;189(4):1535–9.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  10. Akhyani M, Chams-Davatchi C, Naraghi Z, Daneshpazhooh M, Toosi S, Asgari M, Malekhami F. Cervicovaginal involvement in pemphigus vulgaris: a clinical study of 77 cases. Br J Dermatol. 2008;158(3):478–82.

    Article  CAS  PubMed  Google Scholar 

  11. Malik M, Ahmed AR. Involvement of the female genital tract in pemphigus vulgaris. Obstet Gynecol. 2005;106(5 Pt 1):1005–12.

    Article  PubMed  Google Scholar 

  12. Batta K, Munday PE, Tatnall FM. Pemphigus vulgaris localized to the vagina presenting as chronic vaginal discharge. Br J Dermatol. 1999;140(5):945–7.

    Article  CAS  PubMed  Google Scholar 

  13. Kanawa S, Hashimoto T, Nishikawa T, Nishiyama S. Changes in clinical features, histologic findings, and antigen profiles with development of pemphigus foliaceus from pemphigus vulgaris. Arch Dermatol. 1994;130(12):1534–8.

    Article  Google Scholar 

  14. Zaraa I, Sellami A, Bouguerra C, Sellami MK, Chelly I, Zitouna M, et al. Pemphigus vegetans: a clinical, histological, immunopathological and prognostic study. J Eur Acad Dermatol Venereol. 2011;25(10):1160–7.

    Article  CAS  PubMed  Google Scholar 

  15. Wong KT, Wong KK. A case of acantholytic dermatosis of the vulva with features of pemphigus vegetans. J Cutan Pathol. 1994;21(5):453–6.

    Article  CAS  PubMed  Google Scholar 

  16. Zhang X, Hyjek E, Soltani K, Petronic-Rosic V, Shea CR. Immunohistochemistry for immunoglobulin G4 on paraffin sections for the diagnosis of pemphigus. Arch Pathol Lab Med. 2012;136(11):1402–7.

    Article  PubMed  Google Scholar 

  17. Alexandru A, Zurac S, Salavastru CM, Andrei R, Tebeica T, Staniceanu F, Tiplica GS. Direct immunofluorescence on hair follicles – present and future perspectives. Am J Dermatopathol. 2013;35(4):472–6.

    Article  PubMed  Google Scholar 

  18. Lewis FM, Agarwal A, Neill SM, Calonje JE, Stefanato CM. The spectrum of histopathologic patterns secondary to the application of EMLA on vulvar epithelium: clinicopathologic correlation in three cases. J Cutan Pathol. 2013;40(8):708–13.

    Article  PubMed  Google Scholar 

  19. Chorzelski TP, Kudejko J, Jablonska S. Is papular acantholytic dyskeratosis of the vulva a new entity? Arch Dermatol. 1984;6(6):557–60.

    CAS  Google Scholar 

  20. Cooper PH. Acantholytic dermatosis localized to the vulvocrural area. J Cutan Pathol. 1989;16(2):81–4.

    Article  PubMed  Google Scholar 

  21. Pestereli HE, Karaveli S, Oztekin S, Zorlu G. Benign persistent papular acantholytic and dyskeratotic eruption of the vulva: a case report. Int J Gynecol Pathol. 2000;19(4):374–6.

    Article  CAS  PubMed  Google Scholar 

  22. Saenz AM, Cirocco A, Avendano M, Gonzalez F, Sardi JR. Papular acantholytic dyskeratosis of the vulva. Pediatr Dermatol. 2005;22(3):237–9.

    Article  PubMed  Google Scholar 

  23. Coppola G, Muscardin LM, Piazza P. Papular acantholytic dyskeratosis. Am J Dermatopathol. 1986;8(4):364–5.

    Article  CAS  PubMed  Google Scholar 

  24. Pernet C, Bessis D, Savignac M, Tron E, Guillot B, Hovnanian A. Genitoperineal papular acantholytic dyskeratosis is allelic to Hailey-Hailey disease. Br J Dermatol. 2012;167(1):210–2.

    Article  CAS  PubMed  Google Scholar 

  25. Lipoff JB, Mudgil AV, Young S, Chu P, Cohen SR. Acantholytic dermatosis of the crural folds with ATP2C1 mutation is a possible variant of Hailey-Hailey disease. J Cutan Med Surg. 2009;13(3):151–4.

    CAS  PubMed  Google Scholar 

  26. Logan E, Baxter ML, Walsh NM. White papules and plaques on the vulvar and perineal areas: challenge. Am J Dermatopathol. 2013;35(2):235–6.

    Article  PubMed  Google Scholar 

  27. Burge SM. Hailey-Hailey disease: the clinical presentation, response to treatment and prognosis. Br J Dermatol. 1992;126(3):275–82.

    Article  CAS  PubMed  Google Scholar 

  28. Evron S, Leviatan A, Okon E. Familial benign chronic pemphigus appearing as leukoplakia of the vulva. Int J Dermatol. 1984;23(8):556–7.

    Article  CAS  PubMed  Google Scholar 

  29. Wieselthier JS, Pincus SH. Hailey-Hailey disease of the vulva. Arch Dermatol. 1993;129(10):1344–5.

    Article  CAS  PubMed  Google Scholar 

  30. Misra R, Raman M, Singh N, Agarwal N. Hailey-Hailey disease masquerading as candidiasis. Int J Gynecol Obstet. 1993;42(1):51–2.

    Article  CAS  Google Scholar 

  31. Cockayne SE, Rassl DM, Thomas SE. Squamous cell carcinoma arising in Hailey-Hailey disease of the vulva. Br J Dermatol. 2000;142(3):540–2.

    Article  CAS  PubMed  Google Scholar 

  32. Holst VA, Fair KP, Wilson BB, Patterson JW. Squamous cell carcinoma arising in Hailey-Hailey disease. J Am Acad Dermatol. 2000;43(2 Pt 2):368–71.

    Article  CAS  PubMed  Google Scholar 

  33. Sakuntabhai A, Ruiz-Perez V, Carter S, Jacobsen N, Burge S, Monk S, et al. Mutation in ATP2A2, encoding a Ca2+ pump, cause Darier disease. Nat Genet. 1999;21(3):271–7.

    Article  CAS  PubMed  Google Scholar 

  34. Burge SM, Wilkinson JD. Darier-White disease: a review of the clinical presentation of 193 patients. J Am Acad Dermatol. 1992;27(1):40–50.

    Article  CAS  PubMed  Google Scholar 

  35. Barrett JFR, Murray LA, MacDonald HN. Darier’s disease localized to the vulva. Case report Br J Obstet Gynecol. 1989;96(8):997–9.

    Article  CAS  Google Scholar 

  36. Ridley CM, Buckley CH. Darier’s disease localized to the vulva (correspondence). Br J Dermatol. 1991;98(1):112.

    CAS  Google Scholar 

  37. Vazquez J, Morales C, Gonzalez LO, Lamelas ML, Ribas A. Vulval squamous cell carcinoma arising in localized Darier’s disease. Eur J Obstet Gynecol Reprod Biol. 2002;102(2):206–8.

    Article  PubMed  Google Scholar 

  38. Duray PH, Merino MJ, Axiotis C. Warty dyskeratoma of the vulva. Int J Gynecol Pathol. 1983;2(3):286–93.

    Article  CAS  PubMed  Google Scholar 

  39. Waisbourd-Zinman O, Ben-Amitai D, Cohen AD, Feinmesser M, Mimouni D, Adir-Shani A, et al. Bullous pemphigoid in infancy: clinical and epidemiological characteristics. J Am Acad Dermatol. 2008;58(1):41–8.

    Article  PubMed  Google Scholar 

  40. Hertl M, Schmidt T. Underrecognition of the heterogeneous clinical spectrum of bullous pemphigoid. JAMA Dermatol. 2013;26:1–2.

    Google Scholar 

  41. Bakker CV, Terra JB, Pas HH, Jonkman MF. Bullous pemphigoid as pruritus in the elderly: a common presentation. JAMA Dermatol. 2013;26:1–5.

    Google Scholar 

  42. Schiavo AL, Ruocco E, Brancaccio G, Caccavale S, Ruocco V, Wolf R. Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies. Clin Dermatol. 2013;31(4):391–9.

    Article  PubMed  Google Scholar 

  43. Marren P, Wojnarowska F, Venning V, Wilson C, Nayar M. Vulvar involvement in auto-immune bullous diseases. J Reprod Med. 1993;38(2):101–7.

    CAS  PubMed  Google Scholar 

  44. Urano S. Localized bullous pemphigoid of the vulva. J Dermatol. 1996;23(8):580–2.

    CAS  PubMed  Google Scholar 

  45. Kharfi M, Khaled A, Anane R, Fazaa B, Karnoun MR. Early onset childhood cicatricial pemphigoid: a case report and review of the literature. Pediatr Dermatol. 2010;27(2):119–24.

    Article  PubMed  Google Scholar 

  46. Ridley CM. Cicatricial pemphigoid of the vulva. Am J Obstet Gynecol. 1985;152(7 Pt 1):916–7.

    Article  CAS  PubMed  Google Scholar 

  47. Marren P, Walkden V, Mallon E, Wojnarowska F. Vulval cicatricial pemphigoid may mimic lichen sclerosus. Br J Dermatol. 1996;134(3):522–4.

    Article  CAS  PubMed  Google Scholar 

  48. Farrell AM, Kirtschig G, Dalziel KL, Allen J, Dootson G, Edwards S, Wojnarowska F. Childhood genital pemphigoid: a clinical and immunopathological study of five patients. Br J Dermatol. 1999;140(2):308–12.

    Article  CAS  PubMed  Google Scholar 

  49. Trueb RM, Didierjean L, Fellas A, Elias A, Borradori L. Childhood bullous pemphigoid. Report of a case with characterization of the targeted antigens. J Am Acad Dermatol. 1999;40(2 Pt 2):338–44.

    Article  CAS  PubMed  Google Scholar 

  50. Levine V, Sanchez M, Nestor M. Localised vulvar pemphigoid in a child misdiagnosed as sexual abuse. Arch Dermatol. 1992;128(6):804–6.

    Article  CAS  PubMed  Google Scholar 

  51. Frith P, Charnock M, Wojnarowska F. Cicatricial pemphigoid diagnosed from ocular features in recurrent severe vulval scarring. Two case reports. Br J Obstet Gynaecol. 1991;98(5):482–4.

    Article  CAS  PubMed  Google Scholar 

  52. Chan LS, Ahmed AR, Anhalt GJ, Bernauer W, Cooper KD, Elder MJ, et al. The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenetic factors, medical treatment and prognostic indicators. Arch Dermatol. 2002;138(3):370–9.

    Article  PubMed  Google Scholar 

  53. Liu Z, Diaz LA. Bullous pemphigoid: end of the century overview. J Dermatol. 2001;28(11):647–50.

    Article  CAS  PubMed  Google Scholar 

  54. Arechalde A, Braun RP, Calza AM, Hertl M, Didierjean L, Saurat JH, Borradori L. Childhood bullous pemphigoid associated with IgA antibodies against BP180 or BP230 antigens. Br J Dermatol. 1999;140(1):112–8.

    Article  CAS  PubMed  Google Scholar 

  55. Collier P, Wojnarowska F, Allen J, Kirtschiq G. Molecular overlap of the IgA target antigens in the subepidermal blistering diseases. Dermatology. 1994;189 Suppl 1:105–7.

    Article  PubMed  Google Scholar 

  56. Leverkus M, Bhol K, Hirako Y, Pas H, Sitaru C, Baier G, et al. Cicatricial pemphigoid with circulating autoantibodies to beta4 integrin, bullous pemphigoid 180 and bullous pemphigoid 230. Br J Dermatol. 2001;145(6):998–1004.

    Article  CAS  PubMed  Google Scholar 

  57. Ghohestani RF, Nicolas JF, Rouselle P, Claudy AL. Identification of 168-kDa mucosal antigen in a subset of patients with cicatricial pemphigoid. J Invest Dermatol. 1996;107(1):136–9.

    Article  CAS  PubMed  Google Scholar 

  58. Vassileva S. Drug-induced pemphigoid: bullous and cicatricial. Clin Dermatol. 1998;16(3):379–87.

    Article  CAS  PubMed  Google Scholar 

  59. Pfaltz K, Mertz K, Rose C, Scheidegger P, Pfaltz M, Kempf W. C3d immunohistochemistry on formalin-fixed tissue is a valuable tool in the diagnosis of bullous pemphigoid of the skin. J Cutan Pathol. 2010;37(6):654–8.

    Article  PubMed  Google Scholar 

  60. Korman NJ. Bullous pemphigoid. The latest in diagnosis, prognosis, and therapy. Arch Dermatol. 1998;134(9):1137–41.

    CAS  PubMed  Google Scholar 

  61. Lehman JS, Carnilleri MJ. Diagnostic utility of direct immunofluorescence findings around hair follicles and sweat glands in immunobullous disease. J Cutan Pathol. 2013;40(2):230–5.

    Article  PubMed  Google Scholar 

  62. Lim HW, Bystryn JC. Evaluation and management of disease of the vulva: bullous diseases. Clin Obstet Gynecol. 1978;21(4):1007–22.

    Article  CAS  PubMed  Google Scholar 

  63. Lipozencic J, Ljubojevic S, Bukvic-Mokos Z. Pemphigoid gestationis. Clin Dermatol. 2012;30(1):51–5.

    Article  PubMed  Google Scholar 

  64. Semkova K, Black M. Pemphigoid gestationis: current insights into pathogenesis and treatment. Eur J Obstet Reprod Biol. 2009;145(2):138–44.

    Article  Google Scholar 

  65. Castro LA, Lundell RB, Krause PK, Gibson LE. Clinical experience in pemphigoid gestationis: report of 10 cases. J Am Acad Dermatol. 2006;55(5):823–8.

    Article  PubMed  Google Scholar 

  66. Chorzelski TP, Jablonska S. IgA linear dermatosis of childhood (chronic bullous disease of childhood). Br J Dermatol. 1979;101(5):535–42.

    Article  CAS  PubMed  Google Scholar 

  67. Egan CA, Zone JJ. Linear IgA bullous dermatosis. Int J Dermatol. 1999;38(11):818–27.

    Article  CAS  PubMed  Google Scholar 

  68. Ishiko A, Shimizu H, Masunaga T, Yancey KB, Giudice GJ, Zone JJ, Nishikawa T. 97-kDa linear IgA bullous dermatosis antigen localizes in the lamina lucida between the NC16A and carboxyl terminal domain of the 180 kDa bullous pemphigoid antigen. J Invest Dermatol. 1998;111(1):93–6.

    Article  CAS  PubMed  Google Scholar 

  69. Wojnarowska F, Frith P. Linear IgA disease. Dev Ophthalmol. 1997;28:64–72.

    Article  CAS  PubMed  Google Scholar 

  70. Fortuna G, Salas-Alanis JC, Guidetti E, Marinkovich MP. A critical reappraisal of the current data on drug-induced linear immunoglobulin A bullous dermatosis: a real and separate nosological entity? J Am Acad Dermatol. 2012;66(6):988–94.

    Article  PubMed  Google Scholar 

  71. Leonard JN, Haffenden GP, Ring NP, McMinn RM, Sidqwick A, Mowbray JF, et al. Linear IgA disease in adults. Br J Dermatol. 1982;107(3):301–16.

    Article  CAS  PubMed  Google Scholar 

  72. Petersen CS, Brocks K, Weismann K, Kobayasi T, Thomsen HK. Pretibial epidermolysis bullosa with vulvar involvement. Acta Derm Venereol. 1996;76(1):80–1.

    CAS  PubMed  Google Scholar 

  73. Irvine A, McLean W. Human keratin diseases: the increasing spectrum of disease and subtlety of the phenotype-genotype correlation. Br J Dermatol. 1999;140(5):815–28.

    Article  CAS  PubMed  Google Scholar 

  74. Ishida-Yamamoto A, McGrath J, Chapman S, Leigh IM, Lane EB, Eady RA. Epidermolysis bullosa simplex (Dowling-Meara type) is a genetic disease characterized by an abnormal keratin-filament network involving keratins K5 and K14. J Invest Dermatol. 1991;97(6):959–68.

    Article  CAS  PubMed  Google Scholar 

  75. McGrath J, Kivirikko S, Ciatti S, Moss C, Dunnill GS, Eady RA, et al. A homozygous nonsense mutation in the alpha 3 chain gene of laminin 5 (LAMA3) in Herlitz junctional epidermolysis bullosa: prenatal exclusion in a fetus at risk. Genomics. 1995;29(1):282–4.

    Article  CAS  PubMed  Google Scholar 

  76. Vidal F, Aberdam D, Miquel C, Christiano AM, Pulkkinen L, Uitto J, et al. Integrin beta 4 mutations associated with junctional epidermolysis bullosa with pyloric atresia. Nat Genet. 1995;10(2):229–34.

    Article  CAS  PubMed  Google Scholar 

  77. Christiano A, Greenspan D, Hoffman G, Zhang X, Tamai Y, Lin AN, et al. A missense mutation in type VII collagen in two affected siblings with recessive dystrophic epidermolysis bullosa. Nat Genet. 1993;4(1):62–6.

    Article  CAS  PubMed  Google Scholar 

  78. Nordal EJ, Mecklenbeck S, Hausser I, Skranes J, Bruckner-Tuderman L, Gedde-Dahl Jr T. Generalized dystrophic epidermolysis bullosa: identification of a novel, homozygous glycine substitution, G2031S, in exon 73 of COL7A1 in monozygous triplets. Br J Dermatol. 2001;144(1):151–7.

    Article  CAS  PubMed  Google Scholar 

  79. Apalla Z, Sotiriou E, Lazaridou E, Manousari A, Trigoni A, Papagarifallou I, Ioannides D. Pemphigus vegetans of the tongue: a diagnostic and therapeutic challenge. Int J Dermatol. 2013;53(3):350–1.

    Article  Google Scholar 

  80. Ahmed AR, Blose DA. Pemphigus vegetans. Neumann type and hallopeau type. Int J Dermatol. 1984;23:135–41.

    Article  CAS  PubMed  Google Scholar 

  81. Saruta H, Ishii N, Teye K, Ono F, Ohyama B, Koga H, et al. Two cases of pemphigus vegetans with IgG anti-desmocollin 3 antibodies. JAMA Dermatol. 2013;149(10):1209–13.

    Article  PubMed  Google Scholar 

  82. Storwick SG, Prihoda MB, Fulton JR, et al. Pyodermatitis-pyostomatitis vegetans: a specific marker for inflammatory bowel disease. J Am Acad Dermatol. 1994;31:336–42.

    Article  CAS  PubMed  Google Scholar 

  83. Mascarenhas R, Fernandes B, Reis JP, Tellechea O, Figueiredo A. Pemphigus vulgaris with nail involvement presenting with vegetating and verrucous lesions. Dermatol Online J. 2003;9:14.

    CAS  PubMed  Google Scholar 

  84. Abellaneda C, Mascaro Jr JM, Vazquez MG, Pablo IM, Iranzo P. All that glitters is not pemphigus: pyodermatitis-pyostomatitis vegetans misdiagnosed as IgA pemphigus for 8 years. Am J Dermatopathol. 2011;33(1):e1–6.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Mai P. Hoang

  2. Department of Anatomic Pathology, Hospital Universitari Germans Trias i Pujol, Badalona, Spain

    María Teresa Fernández-Figueras

  3. Department of Dermatology, Brigham and Women’s Hospital, Boston, MA, USA

    Martin C. Mihm Jr.

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  1. Mai P. Hoang
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  2. María Teresa Fernández-Figueras
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  3. Martin C. Mihm Jr.
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Correspondence to Mai P. Hoang .

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Editors and Affiliations

  1. Department of Pathology, Harvard Medical School, Massachusetts General Hospital, Boston, Massachusetts, USA

    Mai P. Hoang

  2. Department of Pathology, Duke University Medical Center, Durham, North Carolina, USA

    Maria Angelica Selim

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Hoang, M.P., Fernández-Figueras, M.T., Mihm, M.C. (2015). Blistering Disorders and Acantholytic Processes Affecting the Epidermis of the Vulva. In: Hoang, M., Selim, M. (eds) Vulvar Pathology. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1807-2_4

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