Cobblestone mouth: an exuberant oral presentation of pemphigus vegetans.

A 45-year-old woman presented with a 4-month history of painful erosions on the oral mucosa and lips, and reported a weight loss of 12 kg due to difficulties in eating. Over the past 2 months she had noticed painful exudative erosions on the inguinal folds and perineum as well as erythematous conjunctiva and easy tearing. She had been prescribed several systemic and topical medications, but her condition had progressively worsened. On physical examination, the surfaces of the vermillion border of the lips, the gums, and the hard palate had multiple white verrucous papules, fissures, erosions, and crusts forming a cobblestone pattern. The dorsum of the tongue had sulci and gyri that gave it a cerebriform appearance (Fig. 1A and B). On the perineum, a well-demarcated erosion with a whitish, exudative surface was seen, in addition to painful, oozing, raised erythematous plaques with a verrucous surface on the inguinal folds (Fig. 1C) and right axilla. The complete skin examination also revealed two small flaccid blisters and an erosion on the back and a superficial erosion on the left lower tarsal conjunctiva. Histological examination of one of the flaccid blisters on the back revealed an intraepidermal blister and suprabasal acantholysis (Fig. 2A), as well as a dense epidermal and dermal inflammatory infiltrate, rich in neutrophils and eosinophils (Fig. 2B). Histological examination of the left inguinal vegetating plaque showed identical findings, in addition to pseudoepitheliomatous epidermal hyperplasia (Fig. 2C). Direct immunofluorescence of perilesional skin showed intercellular staining of the epidermis by immunoglobulin (Ig) G deposits (Fig. 2D). A diagnosis of pemphigus vegetans was established. The patient was treated with systemic prednisolone 60mg/d (1 mg/kg/day) with a slow taper, azathioprine 150mg/d, and topical tacrolimus 1mg/g ointment on the lips. The cutaneous and mucosal lesions regressed completely in two months and the patient was kept on azathioprine 150mg/d. Pemphigus vegetans is a rare variant of pemphigus vulgaris, and is thought to represent a reactive response of the skin to the autoimmune insult of pemphigus vulgaris. It is characterized by flaccid blisters that erupt into erosions and eventually form papillomatous vegetations, especially in intertriginous areas and on the scalp or face. Involvement of the vermilion border of the lips is a clinical hallmark of oral involvement. The tongue may show cerebriform-like changes. The exuberant cobblestone pattern of the lips and oral mucosa associated with marked weight loss in the cur-