Cutaneous Pemphigoid

  • Joost M. MeijerEmail author
  • Jorrit B. Terra


Pemphigoid diseases are a heterogeneous group of subepidermal autoimmune bullous diseases (sAIBDs) that are characterized by autoantibodies against different structural proteins of hemidesmosomes in the epidermal basement membrane zone (EBMZ) (Table. 14.1). Cutaneous pemphigoid includes many subtypes, such as bullous pemphigoid (BP), nonbullous cutaneous pemphigoid (NBCP), Brunsting-Perry cicatricial pemphigoid, lichen planus pemphigoides (LPP), pemphigoid gestationis (PG), anti-p200 pemphigoid, and anti-plectin pemphigoid. Classification of sAIBD subtypes is mainly based on target antigens and/or clinical manifestations. Pathogenesis of cutaneous pemphigoid is mediated by predominantly IgG autoantibodies against different structural proteins in the EBMZ. Diagnosis is based on a combination of clinical criteria, a linear n-serrated deposition pattern along the EBMZ in direct immunofluorescence microscopy and serology. BP is the most common sAIBD and most frequently affects elderly. The incidence of BP increased substantially in the past decades. The clinical manifestations of pemphigoid diseases are heterogeneous. The typical presentation of BP is a severe pruritus with predominantly cutaneous lesions consisting of tense blisters or vesicles, erythema, and urticarial plaques. In NBCP, blistering is completely absent, while pruritus is severe, and papules, plaques, and excoriations may be present. Mucosal lesions develop in 10–20 % of patients. Nikolsky’s sign is negative in pemphigoid. Recommended therapy consists of whole-body application of superpotent topical corticosteroids or oral corticosteroids.


Hemidesmosome Immunoglobulin Basement membrane zone Autoimmune disease Vesiculobullous disease Pemphigoid 


  1. 1.
    Schmidt E, Zillikens D. Pemphigoid diseases. Lancet. 2013;381:320–32.CrossRefPubMedGoogle Scholar
  2. 2.
    Bakker CV, Terra JB, Pas HH, Jonkman MF. Bullous pemphigoid as pruritus in the elderly: a common presentation. JAMA Dermatol. 2013;149:950–3.CrossRefPubMedGoogle Scholar
  3. 3.
    Feliciani C, Joly, P, Jonkman MF, Zambruno G, Zillikens D, Ioannidis D, Kowalewski C, Jedlickova H, Karpati S, Marinovic B, Mimouni D, Uzun S, Yayli S, Hertl M, Borradori L. Management of bullous pemphigoid: the European Dermatology Forum consensus in collaboration with the European Academy of Dermatology and Venereology. Br J Dermatol. 2015;172:867–77.Google Scholar
  4. 4.
    Fairley JA, Burnett CT, Fu CL, Larson DL, Fleming MG, Giudice GJ. A pathogenic role for IgE in autoimmunity: bullous pemphigoid IgE reproduces the early phase of lesion development in human skin grafted to nu/nu mice. J Clin Invest. 2007;127:2605–11.Google Scholar
  5. 5.
    Brunsting LA, Perry HO. Benign pemphigoid; a report of seven cases with chronic, scarring, herpetiform plaques about the head and neck. AMA Arch Derm. 1957;75:489–501.CrossRefPubMedGoogle Scholar
  6. 6.
    Lipozencic J, Ljubojevic S, Bukvic-Mokos Z. Pemphigoid gestationis. Clin Dermatol. 2012;30:51–5.CrossRefPubMedGoogle Scholar
  7. 7.
    Zillikens D, Kawahara Y, Ishiko A, Shimizu H, Mayer J, Rank CV, Liu Z, Giudice GJ, Tran HH, Marinkovich MP, Brocker EB, Hashimoto T. A novel subepidermal blistering disease with autoantibodies to a 200-kDa antigen of the basement membrane zone. J Invest Dermatol. 1996;106:1333–8.CrossRefPubMedGoogle Scholar
  8. 8.
    Goletz S, Hashimoto T, Zillikens D, Schmidt E. Anti-p200 pemphigoid. J Am Acad Dermatol. 2014;71:185–91.CrossRefPubMedGoogle Scholar
  9. 9.
    Buijsrogge JJ, de Jong MC, Kloosterhuis GJ, Vermeer MH, Koster J, Sonnenberg A, Jonkman MF, Pas HH. Antiplectin autoantibodies in subepidermal blistering diseases. Br J Dermatol. 2009;161:762–71.CrossRefPubMedGoogle Scholar

Further Reading

  1. Schmidt E, Zillikens D. Pemphigoid diseases. Lancet. 2013;381:320–32.CrossRefPubMedGoogle Scholar

On the Web

  1. N-versus-u-test: practice serration pattern analysis

Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  1. 1.Department of DermatologyCenter for Blistering Diseases, University Medical Center Groningen, University of GroningenGroningenthe Netherlands

Personalised recommendations