Features of clinical diagnosis of IgA-pemphigus in a patient with a long history of the disease

Abstract

Ige-dependent pemphigus is a rare autoimmune vesicular disease and is characterized by painful and itchy vesicular rashes on the skin. Ige-dependent pemphigus is one of the rarest forms of autoimmune bullous dermatoses. The article describes the clinical case of patient T., aged 82 y.o., who applied to the Novosibirsk Regional Clinical Dermatovenerologic Dispensary with complaints of rashes on the skin of the trunk, limbs, periodically accompanied by itching. For the first time, rashes began to appear 20 years ago, more often in the summer. She was observed by a rheumatologist with a diagnosis of chronic vasculitis, during exacerbation, against the background of therapy with systemic glucocorticosteroids, the rashes regressed. During the last exacerbation after suffering a Novel coronavirus infection at the age of 82 y.о., patient turned to a dermatologist against the background of an injection of a solution with betamethasone, with partially regressed rashes, which made it difficult to establish a diagnosis. After 2 weeks, against the background of discontinuation of therapy, another exacerbation occurred, characterized by obvious clinical manifestations of neutrophilic bullous dermatosis in the form of flaccid pustulеs on the skin of the trunk, upper extremities, as well as serous-purulent crusts arranged in the form of rings and garlands. Based on the history, clinical features and laboratory studies, without the use of immunofluorescence, a provisional diagnosis of IgA-dependent pemphigus was made, systemic glucocorticoid therapy was prescribed with a long-term gradual withdrawal, with a positive effect.