An Atypical Presentation of Pemphigus Vegetans in the Umbilicus

Pemphigus is a chronic, autoimmune bullous disease that affects the skin and mucous membranes. Pemphigus vegetans is a rare variant of pemphigus and presents as oral ulcerations with associated verrucous lesions in intertriginous or flexural areas. A 38-year-old African American woman presented to the clinic with a chief complaint of oral ulcers. She carried a diagnosis of Behcet’s disease and was referred by rheumatology for evaluation of treatment-resistant mucosal ulcerations. At the time of her dermatology visit, she also reported an enlarging umbilical mass that had been present for several months. Further examination of the umbilical lesion identified an exophytic, vegetative mass. Histologic assessment of the lesion identified acanthosis and acantholysis with dermal eosinophils consistent with pemphigus vegetans. A pemphigus antibody panel was done and resulted positive for IgG desmoglein-3 antibodies. The patient was treated with prednisone and rituximab with improvement of her lesions. We present an atypical presentation of pemphigus vegetans involving the umbilicus. This diagnosis should be considered in patients who present with oral erosions and concomitant vegetative lesions, regardless of location or prior diagnoses.