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

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.

Keywords

bullous pemphigoid / direct immunofluorescence / atypical bullous pemphigoid

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