Background. Pemphigus vulgaris (PV) is an autoimmune blistering disease of the skin and mucosa. Anal mucosa may be involved in PV, but the frequency and clinical profile are not fully ascertained. Objective. The aim was to investigate the involvement of the anal area in newly diagnosed PV patients. Patients and Methods. A total of 168 consecutive newly diagnosed PV patients were enrolled. Anal symptoms and signs, involvement of other body sites, and severity of disease were recorded. Results. A total of 47 out of 168 patients (27.9%) had involvement of the anal area. Anal involvement was significantly associated with PV lesions in ophthalmic ( ), nasal ( ), and genital mucosa ( ) but not the oral cavity ( ). There was a significant association between number of involved mucosal sites and anal involvement ( ). Anal involvement was associated with oral severity ( ). Constipation was the most frequent symptom (73.8%) followed by pain on defecation (50%). Seventeen patients (36%) were symptom-free. Erosion was the most frequent sign (91.5%). Conclusion. Anal involvement in PV seems to be more frequent than previously assumed. Routine anal examination is recommended even in asymptomatic patients as anal involvement appears to correlate with the severity of PV. 1. Introduction Pemphigus vulgaris (PV) is a rare, autoimmune, potentially fatal mucocutaneous bullous disease in which pathogenic autoantibodies are directed against the keratinocyte cell surface molecules desmoglein 3 (Dsg3) and to a lesser extent Dsg1 [1]. The incidence of this disease varies from 0.16 to 1.62 cases per 100,000 with increased incidence in Jews, Indians, and middle easterners [2]. PV is characterized by bullae that typically begin in the oral cavity and may spread to involve the skin. Other mucosal surfaces including conjunctiva, nasal mucosa, pharynx, larynx, epiglottis, esophagus, cervix, vagina, and penile mucosa may also be affected in the course of disease [3–8]. Anal involvement may also be seen in PV but its frequency and clinical profile are not fully ascertained yet [9–12]. The aim of this study was to investigate the involvement of the anal area in newly diagnosed PV patients presenting to the Autoimmune Bullous Diseases Research Center (ABDRC), Tehran, Iran, during a 15-month period. 2. Patients and Methods This prospective study included 168 consecutive patients newly diagnosed with PV, attending the ABDRC, between October 2009 and January 2011. The diagnosis of PV was based on the presence of clinical features of the disease, including mucocutaneous bullae and erosions
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