Vestnik dermatologii i venerologiiVestnik dermatologii i venerologiiВестник дерматологии и венерологии0042-46092313-6294Rossijskoe Obschestvo Dermatovenerologov i Kosmetologov111010.25208/vdv554-2020-96-1-45-51CLINICAL CASE REPORTSНАБЛЮДЕНИЕ ИЗ ПРАКТИКИResearch ArticleIgG/IgA-pemphigus — extremely rare blistering autoimmune diseasesIgG/IgA-пузырчатка — редкий аутоиммунный буллезный дерматозKaramovaArfenya E.КарамоваАрфеня Эдуардовна
Russian Federation

Cand. Sci. (Med)., Head of the Department of Dermatology

к.м.н., заведующий отделом дерматологии

karamova@cnikvi.ruZnamenskayaLyudmila F.ЗнаменскаяЛюдмила Федоровна
Russian Federation

Dr. Sci. (Med.), Leading Researcher, Department of Dermatology

д.м.н., ведущий научный сотрудник отдела дерматологии

karamova@cnikvi.ruChikinVadim V.ЧикинВадим Викторович
Russian Federation

Dr. Sci. (Med.), Senior Researcher, Department of Dermatology

д.м.н., старший научный сотрудник отдела дерматологии

karamova@cnikvi.ruMihinaViktoria A.МихинаВиктория Алексеевна
Russian Federation

Dermatovenerologist, Department of clinical dermatology

врач-дерматовенеролог отделения клинической дерматологии

karamova@cnikvi.ruNefedovaMaria A.НефедоваМария Андреевна
Russian Federation

Junior Researcher, Dermatology Department

младший научный сотрудник отдела дерматологии

karamova@cnikvi.ruState Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian FederationФГБУ «Государственный научный центр дерматовенерологии и косметологии» Минздрава России30032020961455130032020Copyright ©; 2020, Karamova A.E., Znamenskaya L.F., Chikin V.V., Mihina V.A., Nefedova M.A.Copyright ©; 2020, Карамова А.Э., Знаменская Л.Ф., Чикин В.В., Михина В.А., Нефедова М.А.2020Karamova A.E., Znamenskaya L.F., Chikin V.V., Mihina V.A., Nefedova M.A.Карамова А.Э., Знаменская Л.Ф., Чикин В.В., Михина В.А., Нефедова М.А.https://creativecommons.org/licenses/by/4.0https://vestnikdv.ru/jour/article/view/1110

Purpose. To present a clinical case of IgG/IgA pemphigus in a 32-year-old woman.

Material and methods. Clinical and laboratory examinations were performed to diagnose the disease: cytological examination of an impression smear from the bottom of the erosion, histological examination of a skin biopsy sample from the lesion, immunofluorescent examination of a biopsy sample of visually accessible unaffected skin.

Results. Clinical manifestations of the disease were similar to Duhring's dermatitis herpetiformis, morphological ones — to pemphigus vulgaris, but immunofluorescent examination of the skin biopsy sample in equal measure revealed deposits of both IgG and IgA in the epidermis, which allowed to determine a diagnosis of IgG/IgA pemphigus. Dapsone therapy at a dose of 100 mg per day led to a significant improvement in the patient's condition.

Conclusion. Diagnosis of bullous dermatoses requires immunofluorescent examination of a biopsy sample of visually accessible unaffected skin. Dapsone was an effective medication for the patient with IgG/IgA pemphigus.

Цель. Представить клинический случай IgG/IgA-пузырчатки у женщины 32 лет.

Материал и методы. Для диагностики заболевания было проведено клиническое и лабораторное обследование: цитологическое исследование мазка-отпечатка со дна эрозии, гистологическое исследование биоптата кожи из очага поражения, иммунофлюоресцентное исследование биоптата видимо непораженной кожи.

Результаты. Клинические проявления заболевания имели сходство с герпетиформным дерматитом Дюринга, морфологические — с вульгарной пузырчаткой, однако при иммунофлюоресцентном исследовании биоптата кожи в эпидермисе были в равной степени выявлены депозиты как IgG, так и IgA, что позволило установить диагноз IgG/IgA-пузырчатки. Терапия дапсоном в дозе 100 мг в сутки привела к значительному улучшению состояния больной.

Заключение. Диагностика пузырных дерматозов требует проведения иммунофлюоресцентного исследования биоптата видимо непораженной кожи. Эффективным препаратом терапии больной IgG/IgA пузырчаткой явился дапсон.

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