International Journal of Dermatology 2007, 46, 1166–1170 © 2007 The International Society of Dermatology 1166 Abstract Background Pemphigus vulgaris (PV) is a rare autoimmune blistering disease of the skin and mucous membranes. It varies in its clinical profile and epidemiologic characteristics in different parts of the world. Objective To determine the clinical features of PV in Iran in a prospective manner. Methods The study included 140 patients with newly registered PV attending our dermatology clinic between January 2003 and June 2004. Results The mean age at the onset of the disease was 41.5 ± 15.7 years, with a female to male ratio of 1.59 : 1. At presentation to our clinic, both skin and mucosal involvement was observed in 95 patients (67.9%). Cutaneous lesions without the involvement of the mucous membranes were seen in nine patients (6.4%), and exclusive mucosal involvement was present in 36 patients (25.7%). The most common initial localization of the disease was the oral cavity, which was involved in 93 patients (77.5%). The most frequent cutaneous and mucosal sites involved were the thorax and oral cavity, respectively. Conclusion Although minor differences were noted, the results of this study are in relatively good agreement with the literature with regard to the age, gender, and initial presentation of PV in Iran. Some skin sites, such as the scalp, thorax, and axilla, may be more commonly affected in men. Patients with initial mucosal lesions were significantly younger than those with initial cutaneous involvement. Mucosal lesions other than the oral mucosa may be more common than previously thought. Blackwell Publishing Ltd Oxford, UK IJD International Journal of Dermatology 0011-9059 © 2007 The International Society of Dermatology XXX Report Pemphigus vulgaris in Iran Esmaili et al. Report Pemphigus vulgaris in Iran: a clinical study of 140 cases Nafiseh Esmaili, MD, Cheyda Chams-Davatchi, MD, Mahin Valikhani, MD, Maryam Daneshpazhooh, MD, Kamran Balighi, MD, Zahra Hallaji, MD, Masoumeh Barzegari, MD, Maryam Akhyani, MD, Zahra S. Ghodsi, MD, Hossein Mrotazavi, MD, Zahra S. Naraghi, MD, and Siavash Toosi, MD From the Pemphigus Research Unit, Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran Correspondence Nafiseh Esmaili, MD Razi Hospital Vahdat-Eslami Square Tehran 119960 Iran E-mail: nafiseesmaili@yahoo.com Introduction Pemphigus vulgaris (PV) is a rare autoimmune blistering disease of the skin and mucous membranes characterized by the presence of autoantibodies targeting desmoglein 3, a surface antigen of keratinocytes, engaged in the maintenance of cell–cell junctions. 1 The incidence of the disease varies in the range 0.07–3.2 cases per 100,000 population per year depending on the geographic location, and is about 1 per 100,000 population in Iran. 2 PV typically runs a chronic course, with blisters, painful erosions, and ulcers on the mucosa and skin. It is a worldwide disease and varies in its presentation, severity, clinical profile, and epidemiologic characteristics in different areas. The purpose of this study was to determine the demography and clinical features of PV in Iran in a pro- spective manner. The characteristics of PV in 140 newly diag- nosed patients during an 18-month period are described. Patients and methods The study included 140 patients with newly registered PV attending the dermatology clinic during the period January 2003 to June 2004 (18 months). The study was carried out at the Pemphigus Research Unit, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran. In all patients enrolled, the diagnosis of PV was based on the typical clinical features of the disease, confirmed by histopathologic and direct immunofluorescence (DIF) findings based on revised diagnostic criteria. 3 Indirect immunofluorescence (IIF), using a substrate of monkey esophagus, was performed in a number of patients. In patients with mucosal lesions only, a biopsy was obtained from intraoral lesions and DIF was performed on perilesional normal mucosa. Only newly diagnosed patients who had not received any immunosuppressive therapy were enrolled in the study. Each patient was subjected to a detailed review of clinical history, similar disease in other family members, drug intake, and a complete physical examination. Various clinical parameters were studied, including age, sex, duration of the disease, anatomic sites affected at the onset of disease and subsequently, number of skin lesions, and size of the largest skin lesion. Statistical analysis was performed using the chi-squared test for differences in proportion and the Mann–Whitney U-test for differences in means. Fisher’s exact test was used when necessary.