DUHRING'S HERPETIFORM DERMATITIS: KEY ASPECTS OF IMMUNOPATHOGENESIS AND THEIR INFLUENCE ON THE CHOICE OF TREATMENT TACTICS

The review is a comprehensive analysis of current scientific data on Duhring's dermatitis herpetiformis. The main attention is paid to an in-depth understanding of the immunopathogenesis of the disease, in which autoantibodies of the IgA class play a key role, primarily directed against epidermal transglutaminase. Duhring's dermatitis is a specific skin manifestation of gluten-dependent enteropathy, which explains its inextricable link with celiac disease. Even in the presence of a classic clinical picture, including polymorphic itchy rashes, verification of the diagnosis requires mandatory morphological confirmation. The "gold diagnostic standard" is the direct immunofluorescence of a biopsy of the affected skin, which makes it possible to detect IgA deposits in the papillary layer of the dermis. Serological diagnostics, including the determination of antibodies to transglutaminase and endomyelium of the IgA class, is a highly sensitive and specific noninvasive method. It is of key importance not only for the confirmation of Duhring's dermatitis, but also for the detection of concomitant celiac disease, which is often asymptomatic or latent. In this regard, the review highlights the need for mandatory screening of all patients with Duhring's dermatitis for celiac disease, including, if indicated, esophagogastroduodenoscopy with biopsy of the small intestine mucosa. The work focuses on current therapy algorithms, which are based on two main strategies.: strict gluten-free diet and prescription of drug therapy. A gluten-free diet is a pathogenetic treatment method that leads to remission of the skin process, a decrease in autoantibody levels, and resolution of intestinal pathology. However, its effect may be delayed, which requires the appointment of drug therapy. The first-line drug for the control of skin manifestations remains dapsone, the effectiveness of which is due to the suppression of neutrophil activity. The review analyzes in detail the schemes of its administration, safety monitoring, including monitoring of the total blood count, glucose-6-phosphate dehydrogenase and methemoglobin levels, as well as potential side effects. For patients with refractory course or dapsone intolerance, the article analyzes generally accepted methods and fundamentally new therapeutic strategies. Among them, rituximab, a monoclonal antibody to CD20, JAK kinase inhibitors, and the interleukin–4 and -13 blocker dupilumab were considered. Of particular practical value of the review is a systematic step–by-step algorithm for patient management that combines all the aspects considered, from primary diagnosis using highly specific serological markers to long-term remission control. This algorithm serves as a ready-made tool for the clinician, allowing him to optimize diagnosis and treatment based on the latest achievements of evidence-based medicine, minimize the risks of therapy and significantly improve the quality of life of patients.

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