Pemphigus Vulgaris Case Study

Case report

A 42-year-old Thai man came to the Institute of Dermatology with intractable stomatitis and itchy rash on perianal area for 1 year. Initially, the cutaneous lesions started with few discrete vesicles and bullae on his face, scalp, right arm and itchy rash on perianal area. Later, the lesions on his scalp and perianal area had gradually progress into jagged mass. The lesions became intensely itchy and occasionally painful without tendency to heal. The patient had no other systemic symptom. He had no underlying disease and denied neither traumatic history nor exposure to chemical compound. Neither similar skin lesions nor malignancy presented in his family members.

Physical examination revealed localized large well-defined papillomatous

Cerebriform tongue is a common sign seen in pemphigus vegetans presented with a typical pattern of sulci and gyri over dorsum of the tongue.2-4 This clinical sign can be used as a clue for the diagnosis of pemphigus vegetans.

Pemphigus vegetans is clinically classified as two variants, which are differentiated based on their clinical presentation and treatment response. 1) Neumann type with periorificial papillomatous vegetations and 2) Hallopeau type with pustular lesions evolving into vegetations preferentially affecting the intertriginous areas and a benign course with few relapses5,6. They can occur over normal skin or over the lesions of pemphigus vulgaris. Half of pemphigus vegetans cases have lesions in the oral cavity months preceding cutaneous lesions. Patients with cutaneous lesions will ultimately develop oral manifestations later. The large plaques on perianal area seen in our patient were typical pattern of sulci, gyri over the flexures seen in pemphigus vegetans. Cerebriform tongue, described as “Premalatha sign”, was found in this patient. In a study of 12 pemphigus

The lesions gradually improved within 3 months and the dosage of prednisolone was slowly decreased to 15 mg/day. 3 months later, the lesions were resolved with flattening of lesions leaving residual post-inflammatory hyperpigmentation and the treatments were gradually adjusted to prednisolone 5 mg/day and azathioprine 50 mg/day to control the symptoms. There was no recurrence after a 6-month follow-up. As maintenance therapy, azathioprine was administered 50 mg on every other day and prednisolone was discontinued. After 12-month treatment, the level of anti-desmoglein 3 antibody was decreased from 483 to