Pemphigus Vegetans -Masquerading as malignancy of lip A Case Report

Abstract

Pemphigus vegetans is a rare variant of pemphigus vulgaris characterized by pustules or papillomatous vegetations, preferentially affecting intertriginous and periorificial areas. Exceptional manifestations may be misdiagnosed resulting in delayed diagnosis and treatment.  We herein report an unusual manifestation of Pemphigus Vegetans in a 58-year-old female presented with history of swelling and pain in the lower lip for 4 months. Suspecting malignancy of lip-edge wedge biopsy of the lesion was done. On examination it was a proliferative lesion of size 2 x 1 cm in the lower lip, irregular in shape with no discharge. On cut section a grey white to grey brown soft tissue fragment measuring 1.5 x 1 x 0.7 cm lesion was received. Microscopic examination of multiple sections showed a polypoidal ulcerative swelling with marked pseudoepitheliomatous hyperplasia, hyperkeratosis, marked irregular acanthosis and a focus showing suprabasal clefting. The base of the supra basal cleft showed villi formation covered by stratum basalis cells giving “tomb stone appearance”. Dermis showed marked inflammatory infiltrates consisting of intraepidermal eosinophils, numerous plasma cells and lymphocytes, along with inflammatory infiltrates in the minor salivary glands. There is no evidence of malignancy. On further evaluation with immunofluorescence intercellular deposition of IgG and C3 in a fishnet pattern was seen confirming the diagnosis of Pemphigus vegetans.