Pemphigus vulgaris presenting as gingival hypertrophy in a child

Abstract

Pemphigus vulgaris is a mucocutaneous autoimmune disease caused by autoantibodies against the desmosomal proteins desmoglein 3 and desmoglein 1. Oral pemphigus vulgaris has rarely been reported to occur in children and adolescents. A current literature review reported 50 cases, 7 of which presented with oral lesions exclusively. Oral lesions of pemphigus commonly present as vesicles or bullae that ulcerate easily and occur most frequently in the buccal, palatal and labial mucosa. Gingival lesions usually present as desquamative gingivitis. We report a case of a healthy 17-year old Hispanic female, taking no medications, who presented with an 8-year history of gingival hypertrophy and no other oral, skin or other mucosal lesions. Clinically she had severe gingival hypertrophy affecting both the facial and lingual surfaces and covering most of the clinical crowns. The surface epithelium appeared eroded in areas and the tissues bled easily. Surgical excision of all hypertrophied gingival tissue was done in the operating room. The final diagnosis on light microscopy was inflammatory hyperplasia and ancantholysis. Direct immunofluorescence showed intercellular deposition of IgG and C3 and indirect immunofluorescence showed IgG positive intercellular staining a 1:80 dilution. Testing for serum antibodies against desmoglein 1 and 3 however was negative. After further evaluation with dermatologist, a diagnosis of atypical pemphigus vulgaris was made. Only 2 other reported cases of pemphigus vulgaris have presented with oral nodules or masses but none exclusive to the gingiva. This is the first documented case of pemphigus vulgaris presenting as gingival hypertrophy in a child without any other mucosal or skin lesions.