Spectrum of autoimmune vesiculobullous diseases in Iran: a 13-year retrospective study

Autoimmune bullous diseases (ABDs) represent a group of rare, acquired disorders characterized by overlapping features with involvement of the skin and mucous membranes, resistance to treatment, and potential lethality that comprise pemphigus, bullous pemphigoid (BP), epidermolysis bullosa, dermatitis herpetiformis, and linear immunoglobulin A bullous dermatosis.

The main aim of this study was to identify the epidemiologic characteristics and clinical courses of these common diseases in Hamadan, Iran. Few surveys have been carried out to demonstrate the whole spectrum of ABDs in the literature. Notably, in Hamadan we are the first.

This 13-year retrospective study was designed to evaluate all of documented data obtained from hospitalized patients with ABDs at Farshchian Hospital from October 1999 to October 2012. We collected information on epidemiologic data, clinical aspects, histologic findings, and therapy prescribed. Data were analyzed using SPSS.

Of 168 patients, 78% had pemphigus. The age of patients at presentation ranged from 1 month to 115 years, with a mean of 47.5±19.93 years. Mucosal or skin involvement of ABDs was statistically significant (P<0.001). The incidence of ABDs differed significantly based on anatomic location (P=0.003). We documented three deaths.

Compared to previous literature, our findings showed equal epidemiologic properties in Iran. Although pemphigus was the most common ABD followed by BP, it is expected that in line with the global trend, an increase in BP will be driven by population aging in Iran.