Incidence of autoimmune bullous diseases in Serbia: a 20-year retrospective study.

BACKGROUND AND OBJECTIVES: While most previous surveys on the clinico-epidemiological features of autoimmune bullous diseases (AIBDs) have predominantly focused on a single disease entity or just one disease group, there have been only few studies examining the incidence of various AIBDs. In the present study, we set out to determine the spectrum of AIBDs, to estimate the incidence of the most common AIBDs, and to examine their temporal trends in Central Serbia over a period of 20 years. METHODS: We retrospectively recruited 1,161 new AIBD cases diagnosed in Central Serbia during the period from January 1991 to December 2010. The diagnosis was based on strict clinical, histological, and immunohistological evaluation. RESULTS: The incidence rates were: 4.35 per million population/year (pmp/year) for pemphigus, 4.47 pmp/year for pemphigoid, 1.42 pmp/year for dermatitis herpetiformis (DH), 0.25 pmp/year for linear IgA disease, and 0.08 pmp/year for epidermolysis bullosa acquisita. In the period observed, age-adjusted incidence rates significantly increased for pemphigus and particularly for pemphigoid, whereas they decreased, albeit not significantly, for DH. CONCLUSIONS: For the first time, our study evaluates the incidence rates of the entire spectrum of AIBDs in Serbia, and examines their temporal trends over a 20-year period. To the best of our knowledge, our finding of similar incidence rates for pemphigus and pemphigoid has previously not been reported.

Pemphigus herpetiformis - a case report of a rare form of pemphigus and review of the literature.

Introduction: Pemphigus herpetiformis is the rare variant of pemphigus with characteristic clinical features, histopathological findings different from the convectional pemphigus, and immunological findings consistent with pemphigus. Case report: We presented a 65-year-old woman with initial pruritus followed by pruritic urticarial papules and plaques, some with annular rings of tense vesicles on the periphery, on the trunk and extremities, with no mucous lesions. Histopathological examination demonstrated spongiosis and intraepidermal vesicles in the mid or subcorneal epidermis in some biopsy specimen, with neutrophil and eosinophil infiltrate. Direct immunoflorescent microscopy revealed intercellular IgG deposition, most prominent in the upper layers of epidermis. Indirect immunoflorescent microscopy showed intercellular binding of IgG autoantibodies in the patient's sera. Initially the patient was threated with systemic corticosteroids and azathioprine, but dapson provided complete clinical remission. Conclusion: This entity was established 40 years ago, and around 100 patients have been reported worldwide. It is important to be aware of this particular form of pemphigus because clinical presentation, course of the disease and therapeutic approach are different from conventional forms of pemphigus.