Immunomorphological insights into clinical variants of bullous pemphigoid in Moscow Region patients

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Abstract

Bullous pemphigoid is an autoimmune blistering dermatosis in which the pathogenesis is primarily characterized by the production of autoantibodies IgG and IgE against hemidesmosomal proteins (BP180 and BP230) and the disruption of T-cell immune response regulation.

The classical understanding of the clinical course of bullous pemphigoid has undergone significant transformation in recent years due to the identification of numerous conditions that do not exhibit typical clinical presentations but are diagnosed as bullous pemphigoid based on immunofluorescence’s results. Often, when an atypical clinical form of bullous pemphigoid develops, a specialist physician may not have grounds to include bullous pemphigoid in the differential diagnosis. Consequently, specific diagnostic tests (such as immunomorphological examinations of skin biopsies) that would allow for the verification of this blistering dermatosis are not performed for the patient. Clearly, due to the absence of an accurate diagnosis, patients do not receive the necessary therapy. There is currently no information in the world literature regarding the frequency of atypical forms of bullous pemphigoid.

However, it is known that bullous pemphigoid is a relatively common blistering dermatosis in the population of European countries, with a prevalence ranging from 2.4 to 66 cases per million people, depending on the region.

About the authors

Anthon V. Molochkov

Moscow Regional Research and Clinical Institute; Peoples' Friendship University of Russia

Email: antmd@yandex.ru
ORCID iD: 0000-0002-6456-998X
SPIN-code: 8853-5050

MD, Dr. Sci. (Med.), Professor

Russian Federation, Moscow; Moscow

Yulia V. Molochkova

Moscow Regional Research and Clinical Institute

Email: yulia.molochkova@yandex.ru
ORCID iD: 0000-0001-9021-6494
SPIN-code: 2051-0250

MD, Dr. Sci. (Med.), Associate Professor

Russian Federation, Moscow

Maria K. Monaenkova

Moscow Regional Research and Clinical Institute

Author for correspondence.
Email: mar.monaenkova@gmail.com
ORCID iD: 0000-0001-8479-1978
SPIN-code: 9662-8661
Russian Federation, Moscow

Alina A. Guseva

Moscow Regional Research and Clinical Institute

Email: aag-alina@yandex.ru
ORCID iD: 0000-0002-0825-8802
Russian Federation, Moscow

Vladimir A. Molochkov

Moscow Regional Research and Clinical Institute

Email: vmolochkov@yandex.ru
ORCID iD: 0000-0003-3388-9224
SPIN-code: 2215-1337

MD, Dr. Sci. (Med.), Professor

Russian Federation, Moscow

Oleg V. Karzanov

Moscow Regional Research and Clinical Institute

Email: dr_karzanov@mail.ru
ORCID iD: 0000-0002-2461-546X
SPIN-code: 1196-5829

MD, Cand. Sci. (Med.)

Russian Federation, Moscow

Marina A. Gureeva

Peoples' Friendship University of Russia

Email: marina.gureeva@mail.ru
ORCID iD: 0000-0001-8212-6210
SPIN-code: 6226-9486
Russian Federation, Moscow

Anna G. Kupriyanova

Moscow Regional Research and Clinical Institute

Email: annak2003@bk.ru
ORCID iD: 0000-0002-4538-8075
SPIN-code: 8612-8897

MD, Cand. Sci. (Med.)

Russian Federation, Moscow

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2. Fig. 1. Classic variant of bullous pemphigoid. On the skin of the upper limb there are numerous tense blisters with turbid content, rounded outlines, some of them are located on an erythematous background, numerous erosions with epithelial fragments along the edges, some of the erosions with serous-haemorrhagic crusts on the surface are also visible.

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3. Fig. 2. Erythrodermic form of bullous pemphigoid.

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4. Fig. 3. Pruriginous form of bullous pemphigoid. Numerous papulovesicles resembling elements of prurigo are found on the skin of the trunk and upper limb.

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5. Fig. 4. Seborrhoeic variant of bullous pemphigoid. The skin of the chest shows congestive reddish-bluish widespread, confluent patches with massive, oily, dirty yellow crusts on the surface.

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