Anal Involvement in Pemphigus Vularis

Abstract

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 (P = 0.03), nasal (P = 0.02), and genital mucosa (P < 0.001) but not the oral cavity (P = 0.24). There was a significant association between number of involved mucosal sites and anal involvement (P < 0.001). Anal involvement was associated with oral severity (P = 0.02). 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.

Anal involvement in patient with pemphigus vulgaris: Case report

“…This last one also showed an association with oral mucosa bulla in 87.2 % of patients with anal involvement, as well as a 48 %, 34 %, and 32 % for genital, nasal, and ophthalmic involvement respectively. It has been described to be predominantly associated with late manifestations of the disease or a sign of severity, especially in oral mucosa and, although studies that have characterized these associations have had a limited number of participants, they report similar results [ 3 , 8 ].…”

Beware of anal fissures: pemphigus vulgaris of the anal canal

“…Chams-Davatchi et al [5] observed anal lesions in 2% of 1209 patients with PV, while Malik et al [7] reported an incidence of 10% in their retrospective cohort of 160 PV patients. Kherzi et al [6], in a prospective study of 168 newly diagnosed cases of PV, found a 27.9% rate of anal involvement and suggested that anal PV seems underreported. Anal involvement was significantly associated with PV in other mucosal sites (ophthalmic, nasal, genital).…”

“…Anal involvement was significantly associated with PV in other mucosal sites (ophthalmic, nasal, genital). It was also significantly correlated with the severity of concomitant oral PV, but not with the severity of cutaneous lesions [6]. Hemorrhoids were observed in 17 (10%) patients; interestingly, 16 of the 17 patients with hemorrhoids also had anal PV, suggesting that repeated trauma during defecation might be a triggering factor.…”

“…The oral cavity is the initial site of involvement in up to 70% of patients [3] and involvement of other mucous membranes lined by stratified squamous epithelium is possible: nasal, laryngo-esophageal, conjunctival, genital or anal [4,5]. Although anal involvement has been suggested in 2-27% of PV patients [5,6], there is generally little recognition of its importance or experience of this manifestation among physicians, including dermatologists and gastroenterologists. Several cases may initially be misdiagnosed and improperly treated.…”