Although the oral mucosa is a common site of presentation of pemphigus vulgaris, involvement of the larynx and esophagus is rare [1, 2]. We describe a patient who presented with odynophagia and hoarseness that were found to be caused by laryngeal pemphigus, which may have been triggered by enalapril, a powerful in-vitro acantholytic agent.

A 63-year-old man presented with a 3-week history of odynophagia and hoarseness. For several months he had received enalapril (10 mg orally, once daily) for treatment of hypertension, and he was receiving no other medication. His skin was intact, but severe friability of the oral mucosa and several erosions and ulcerations were noted. Indirect laryngoscopy showed erosions of the pharynx, larynx, and esophagus. Direct laryngoscopy showed marked edema of the supraglottic larynx and extensive inflammatory changes of the pharynx, larynx, and esophagus. Examination of multiple biopsy specimens showed an inflammatory infiltrate of the laryngeal submucosa with prominent acantholysis of the overlying epithelium and formation of suprabasal clefts. These changes were consistent with laryngeal pemphigus. Immunofluorescence showed epithelial intercellular immunoreactivity for IgG and C3 without reactivity for IgM or IgA, which supported the diagnosis of pemphigus. The patient was treated with oral prednisone and responded well, and the mucosal lesions resolved within 3 weeks.

Drug-induced pemphigus is a well-recognized entity most commonly associated with drugs that contain thiol, such as penicillamine [3]. Only a few cases of pemphigus that appeared to be triggered by nonthiol drugs have been reported (Table 1). Among the latter are three cases of enalapril-associated pemphigus [4]; however, isolated mucosal involvement has not been previously described. De Angelis and colleagues [5] recently reported experimental evidence that enalapril, although not a thiol, is a powerful in vitro acantholytic agent. Physicians should be aware of uncommon presentations of pemphigus vulgaris and its occurrence as a potential complication of therapy with frequently used medications such as enalapril.