Bullous pemphigoid. Сlinic, Diagnosis and Treatment

Cover Page

Cite item


The article describes modern views on the predisposing factors, features, immunological changes and link with the histocompatibility antigens HLA, the role of circulating autoantibodies, cytokines, chemokines, matrix metalloproteinases and BP180 (BPAg2) и BP230 (BPAg1), those role in the pathogenesis of the bullous pemphigoid and their correlation with the activity of pathological process in patients with bullous pemphigoid. Described experimental model of the disease in animals, modern medications and methods of treatment of this disease.

About the authors

M. B. Drozhdina

Kirov State Medical University, Ministry of Health of the Russian Federation

Author for correspondence.
Email: drozhdina@yandex.ru

D.Sci. (Medicine), Associate Professor, Department of Dermatovenereology and Cosmetology

Russian Federation

S. V. Koshkin

Kirov State Medical University, Ministry of Health of the Russian Federation

Email: koshkin_sergei@mail.ru

D.Sci. (Medicine), Prof., Head of the Department of Dermatovenereology and Cosmetology

Russian Federation


  1. Chan K., Ho H., Kralg E. et al. Bullous Pemphigoid: a 7-Year Survey on 75 Chinese Patients in Hong Kong. Hong Kong J. Dermatol. Venereol. 2006;14(2):71–74.
  2. Gajic-Veljic M., Nikolic M., Medenica L. Juvenile Bullous Pemphigoid: the Presentation and Follow-Up of Six Cases. J. Eur. Acad. Dermatol. Venereol. 2010;24(1):69–72.
  3. Guidelines for the Management of Bullous Pemphigoid. British Association of Dermatologists, 2012.
  4. Langan S. M., Smeeth L., Hubbard R. et al. Bullous Pemphigoid and Pemphigus Vulgaris — Incidence and Mortality in the UK: Population Based Cohort Study. BMJ. 2008;337:a180.
  5. Федеральные клинические рекомендации. Дерматовенерология 2015: Болезни кожи. Инфекции, передаваемые половым путем. 5-е изд., перераб. и доп. М.: Деловой экспресс, 2016. [Federal Clinical Recommendations. Dermatovenerology of 2015: Skin Diseases. Sexually Transmitted Infections. 5th ed., revised and enlarged. M.: Business Express, 2016.]
  6. Iwasaki T., Olivry T., Lapiere J. C. et al. Canine Bullous Pemphigoid (BP): Identification of the 180-kd Canine BP Antigen by Circulating Autoantibodies. Vet. Pathol. 1995;32(4):387–393.
  7. Xu L., O’Toole E. A., Olivry T. et al. Molecular Cloning of Canine Bullous Pemphigoid Antigen 2 cDNA and Immunomapping of NC16A Domain by Canine Bullous Pemphigoid Autoantibodies. Biochim. Biophys. Acta. 2000;1500(1):97–107.
  8. Olivry T., Borrillo A. K., Xu L. et al. Equine Bullous Pemphigoid IgG Autoantibodies Target Linear Epitopes in the NC16A Ectodomain of Collagen XVII (BP180, BPAG2). Vet. Immunol. Immunopathol. 2000;73(1):45–52.
  9. Olivry T., Mirsky M. L., Singleton W. et al. A Spontaneously Arising Porcine Model of Bullous Pemphigoid. Arch. Dermatol. Res. 2000;292(1):37–45.
  10. Chen R., Ning G., Zhao M. L. et al. Mast Cells Play a Key Role in Neutrophil Recruitment in Experimental Bullous Pemphigoid. J. Clin. Invest. 2001;108(8):1151–1158.
  11. Hall R. P. 3rd, Murray J. C., McCord M. M. et al. Rabbits Immunized with a Peptide Encoded for by the 230-kD Bullous Pemphigoid Antigen cDNA Develop an Enhanced Inflammatory Response to UVB Irradiation: a Potential Animal Model for Bullous Pemphigoid. J. Invest. Dermatol. 1993;101(1):9–14.
  12. Liu Z., Giudice G. J., Swartz S. J. et al. The Role of Complement in Experimental Bullous Pemphigoid. J. Clin. Invest. 1995;95(4):1539–1544.
  13. Ujiie H., Shibaki A., Nishie W. et al. A Novel Active Mouse Model for Bullous Pemphigoid Targeting Humanized Pathogenic Antigen. J. Immunol. 2010;184(4):2166–2174.
  14. Olivry T., Chan L. S., Xu L. et al. Novel Feline Autoimmune Blistering Disease Resembling Bullous Pemphigoid in Humans: IgG Autoantibodies Target the NC16A Ectodomain of Type XVII Collagen (BP180/ BPAG2). Vet. Pathol. 1999;36(4):328–335.
  15. Iwata H., Kamio N., Aoyama Y. et al. IgG from Patients with Bullous Pemphigoid Depletes Cultured Keratinocytes of the 180-kDa Bullous Pemphigoid Antigen (Type XVII Collagen) and Weakens Cell Attachment. J. Invest. Dermatol. 2009;129(4):919–926.
  16. Sitaru C., Schmidt E., Petermann S. et al. Autoantibodies to Bullous Pemphigoid Antigen 180 Induce Dermal-Epidermal Separation in Cryosections of Human Skin. J. Invest. Dermatol. 2002;118(4):664–671.
  17. Schmidt E., Obe K., Brocker E. B., Zillikens D. Serum Levels of Autoantibodies to BP180 Correlate with Disease Activity in Patients with Bullous Pemphigoid. Arch. Dermatol. 2000;136(2):174–178.
  18. Zhou X. P., Liu B., Xu Q. et al. Serum Levels of Immunoglobulins G1 and G4 Targeting the Non-Collagenous 16A Domain of BP180 Reflect Bullous Pemphigoid Activity and Predict Bad Prognosis. J. Dermatol. 2016;43(2):141–148.
  19. Kiss M., Perenyi A., Marczinovits I. et al. Autoantibodies to Human Alpha6 Integrin in Patients with Bullous Pemphigoid. Ann. N. Y. Acad. Sci. 2005;1051:104–110.
  20. Bekou V., Thoma-Uszynski S., Wendler O. et al. Detection of Laminin 5-Specific Auto-Antibodies in Mucous Membrane and Bullous Pemphigoid Sera by ELISA. J. Invest. Dermatol. 2005;124(4):732–740.
  21. Frezzolini A., Cianchini G., Ruffelli M. et al. Interleukin-16 Expression and Release in Bullous Pemphigoid. Clin. Exp. Immunol. 2004;137(3):595– 600.
  22. Echigo T., Hasegawa M., Shimada Y. et al. Both Th1 and Th2 Chemokines are Elevated in Sera of Patients with Autoimmune Blistering Diseases. Arch. Dermatol. Res. 2006;298(1):38–45.
  23. Niimi Y., Pawankar R., Kawana S. Increased Expression of Matrix Metalloproteinase-2, Matrix Metalloproteinase-9 and Matrix Metalloproteinase-13 in Lesional Skin of Bullous Pemphigoid. Int. Arch. Allergy Immunol. 2006;139(2):104–113.
  24. Stausbol-Gron B., Deleuran M., Sommer Hansen E., Kragballe K. Development of Bullous Pemphigoid During Treatment of Psoriasis with Adalimumab. Clin. Exp. Dermatol. 2009;34(7):e285–e286.
  25. Eskin-Schwartz M., Drozhdina M., Sarig O. et al. Epidermolytic Ichthyosis Sine Epidermolysis. Am J Dermatopathol. 2017;39(6):440–444.
  26. Wong S. S., Rajakulendran S., Chow E. Bullous Pemphigoid Associated with Carcinoma of the Rectum. J. Hong Kong Geriatr. Soc. 1996;7(1):14–17.
  27. Jemal A., Bray F., Center M. M. et al. Global Cancer Statistics. CA Cancer J. Clin. 2011;61(2):69–90..
  28. Григорьев Д. В. Буллезный пемфигоид Левера. РМЖ. 2014;(8):598. [Grigor’ev D. V. Lever Bullous pemphigoid. 2014;(8):598.]
  29. Waisbourd-Zinman O., Ben-Amitai D., Cohen A. D. et al. Bullous Pemphigoid in Infancy: Clinical and Epidemiologic Characteristics. J. Am. Acad. Dermatol. 2008;58(1):41–48.
  30. Terra J. B., Potze W. J., Jonkman M. F. Whole Body Application of a Potent Topical Corticosteroid for Bullous Pemphigoid. J. Eur. Acad. Dermatol. Venereol. 2014;28(6):712–718.
  31. Gual A., Iranzo P., Mascaro´ J. M. Jr. Treatment of Bullous Pemphigoid with Low-Dose Oral Cyclophosphamide: a Case Series of 20 Patients. J. Eur. Acad. Dermatol. Venereol. 2014;28(6):814–818.
  32. Iwata Y., Komura K., Kodera M. et al. Correlation of IgE Autoantibody to BP180 with a Severe Form of Bullous Pemphigoid. Arch. Dermatol. 2008;144(1):41–48.
  33. Morrison L. H. Therapy of Refractory Pemphigus Vulgaris with Monoclonal Anti-CD20 Antibody (Rituximab). J. Am. Acad. Dermatol. 2004;51(5):817–819.
  34. Ahmed A. R., Spigelman Z., Cavacini L. A., Posner M. R. Treatment of Pemphigus Vulgaris with Rituximab and Intravenous Immune Globulin. N. Engl. J. Med. 2006;355(17):1772–1779.
  35. Dupuy A., Viguier M., Bedane C. et al. Treatment of Refractory Pemphigus Vulgaris with Rituximab (Anti-CD20 Monoclonal Antibody). Arch. Dermatol. 2004;140(1):91–96.
  36. Reguiaї Z., Tchen T., Perceau G., Bernard P. Efficacy of Rituximab in a Case of Refractory Bullous Pemphigoid. Ann. Dermatol. Venereol. 2009;136(5):431–434.
  37. Balakirski G., Alkhateeb A., Merk H. F. et al. Successful Treatment of Bullous Pemphigoid with Omalizumab as Corticosteroid-Sparing Agent: Report of Two Cases and Review of Literature. J. Eur. Acad. Dermatol. Venereol. 2016;30(10):1778–1782.
  38. Cugno M., Marzano A. V., Bucciarelli P. et al. Increased Risk of Venous Thromboembolism in Patients with Bullous Pemphigoid. The INVENTEP (INcidence of VENous ThromboEmbolism in bullous Pemphigoid) Study. Thromb. Haemost. 2016;115(1):193–199.
  39. Langan S. M., Groves R. W., West J. The Relationship Between Neurological Disease and Bullous Pemphigoid: a Population-Based Case-Control Study. J. Invest. Dermatol. 2011;131(3):631–636.
  40. Taghipour K., Chi C. C., Bhogal B. et al. Immunopathological Characteristics of Patients with Bullous Pemphigoid and Neurological Disease. J. Eur. Acad. Dermatol. Venereol. 2014;28(5):569–573.
  41. Jedlickova H., Hlubinka M., Pavlik T. et al. Bullous Pemphigoid and Internal Diseases — A Case-Control Study. Eur. J. Dermatol. 2010;20(1):96– 101.
  42. Bernard P., Reguiai Z., Tancrede-Bohin E. et al. Risk Factors for Relapse in Patients with Bullous Pemphigoid in Clinical Remission: a Multi-center, Prospective, Cohort Study. Arch. Dermatol. 2009;145(5):537–542.
  43. Barrick B. J., Ida C. M., Laniosz V. et al. Bullous Pemphigoid, Neurodegenerative Disease, and Hippocampal BP180 Expression: A Retro spective Postmortem Neuropathologic Study. J. Invest. Dermatol. 2016;136(10):2090–2092.

Copyright (c) 2017 Drozhdina M.B., Koshkin S.V.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

СМИ зарегистрировано Федеральной службой по надзору в сфере связи, информационных технологий и массовых коммуникаций (Роскомнадзор).
Регистрационный номер и дата принятия решения о регистрации СМИ: серия ПИ № ФС 77 - 60448 от 30.12.2014.

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies