Report Spectrum of autoimmune bullous diseases in Iran: a 10-year review Maryam Daneshpazhooh 1 , MD, Cheyda Chams-Davatchi 1 , MD, Pooya Payandemehr 1 , MD, Saman Nassiri 1 , MD, Mahin Valikhani 1 , MD, and Zahra Safai-Naraghi 2 , MD 1 Bullous Diseases Research Center, Department of Dermatology, and 2 Department of Dermatopathology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran Correspondence Maryam Daneshpazhooh, MD Bullous Diseases Research Center Department of Dermatology Razi Hospital 11996 Tehran Iran E-mail: daneshpj@tums.ac.ir Funding: None. Conflicts of interest: None. Abstract Background 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 pemphi- gus, based on the level of blister formation. Although they occur worldwide, the relative fre- quencies 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. Objectives This study aimed to assess the relative frequencies of different ABDs in patients presenting to the Bullous Diseases Research Center, Tehran, Iran. Methods 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. Results A total of 1402 patients were diagnosed with ABD during the study period. Pem- phigus 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 immunoglobu- lin 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. Conclusions 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. Introduction 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 tar- get antigens. These diseases can be divided into two major subgroups represented by subepidermal autoim- mune bullous disorders (SABDs) and pemphigus, based on the level of blister formation. Subepidermal ABDs encompass bullous pemphigoid (BP), epidermolysis bull- osa acquisita (EBA), mucous membrane pemphigoid (MMP), pemphigoid gestationis (PG), linear immunoglob- ulin A (IgA) dermatosis (LAD), dermatitis herpetiformis, lichen planus pemphigoides, and bullous systemic lupus erythematosus (BSLE). The pemphigus group comprises pemphigus vulgaris (PV) and its variant, pemphigus vege- tans, superficial pemphigus [pemphigus foliaceus (PF) and pemphigus erythematosus (PE)], paraneoplastic pemphi- gus (PNP), IgA pemphigus, and drug-induced pemphigus. Pemphigus is known to be especially common in Iran. 1–4 However, there has not yet been any study of the relative 35 ª 2012 The International Society of Dermatology International Journal of Dermatology 2012, 51, 35–41