Spectrum of autoimmune bullous diseases in Iran: a 10-year review

Autoimmune bullous diseases (ABDs) are potentially devastating bullous dermatoses of the skin and mucosae characterized by the presence of tissue-bound and circulating antibodies directed against disease-specific target antigens. These diseases comprise two major subgroups of subepidermal autoimmune bullous disorders and pemphigus, based on the level of blister formation. Although they occur worldwide, the relative frequencies of different ABDs show wide geographical variation. Most epidemiological studies on ABDs have focused on single diseases or a group of diseases; published surveys studying the whole spectrum of diseases are scarce.

This study aimed to assess the relative frequencies of different ABDs in patients presenting to the Bullous Diseases Research Center, Tehran, Iran.

Medical files for all newly diagnosed patients with ABD presenting to the Center between March 1997 and February 2006 were examined. Patients with dermatitis herpetiformis were not included.

A total of 1402 patients were diagnosed with ABD during the study period. Pemphigus vulgaris (PV) was the most common ABD (81.2%), followed by bullous pemphigoid (BP) (11.6%), pemphigus foliaceus (PF) (4.4%), pemphigoid gestationis (0.7%), mucous membrane pemphigoid (0.7%), epidermolysis bullosa acquisita (0.5%), linear immunoglobulin A (IgA) disease (0.4%), paraneoplastic pemphigus (0.2%), IgA pemphigus (0.2%), and pemphigus erythematosus (0.1%). The mean age at diagnosis and male : female ratio were 43.4 years and 1 : 1.39, 42.2 years and 1 : 1.34, and 59.4 years and 1 : 1.36 for PV, BP, and PF, respectively.

Pemphigus vulgaris was the most frequent ABD according to our study; cases of PV outnumbered those of BP by a ratio of almost 8 : 1. This finding contrasts with those of studies conducted in Western European countries, in which BP predominates. There was a female predominance in most subtypes of ABD. Mean age at onset of PV and BP was lower than in Europe. In view of its large population of PV patients, Iran should be considered a suitable field for future clinical trials.