Abstract

Pemphigus Vulgaris (PV) is the most common subtype of pemphigus, a rare group of autoimmune bullous diseases affecting the skin and mucous membranes. PV can be further subdivided into mucocutaneous and mucosal dominant types, depending on the extent of cutaneous involvement. Almost all cases of PV have mucosal involvement; however, a rare variant of cutaneous-only PV has been reported in the literature. To our knowledge, only two previous accounts of unilesional scalp PV have been reported. We present an unusual case of cutaneous-only PV involving the scalp.

Highlights

  • Pemphigus represents a rare group of autoimmune bullous diseases affecting the skin and mucous membranes with an incidence of 1 to 16 per million annually.[1,2] Pemphigus Vulgaris (PV) is the most common subtype, comprising 70% of all cases of pemphigus and can be further subdivided into mucocutaneous and mucosal dominant types, depending on the extent of cutaneous involvement.[1]

  • We present a case of cutaneous-only PV involving the scalp

  • A 36-year-old otherwise healthy Caucasian woman presented to the dermatology office with a painful, pruritic, erythematous plaque isolated to the vertex scalp that had been present for one year

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Summary

INTRODUCTION

Pemphigus represents a rare group of autoimmune bullous diseases affecting the skin and mucous membranes with an incidence of 1 to 16 per million annually.[1,2] Pemphigus Vulgaris (PV) is the most common subtype, comprising 70% of all cases of pemphigus and can be further subdivided into mucocutaneous and mucosal dominant types, depending on the extent of cutaneous involvement.[1]. A 36-year-old otherwise healthy Caucasian woman presented to the dermatology office with a painful, pruritic, erythematous plaque isolated to the vertex scalp that had been present for one year. Minimal improvement was noted on this regimen, and the patient was still experiencing pain and irritation which led her to seek secondary evaluation by a dermatology physician. Physical examination at this time revealed a beefy red, macerated plaque on her vertex scalp with overlying scale (Figure 1). Two 4-mm punch biopsies were performed and sent for histopathological and immunohistochemical analysis. Histopathology revealed suprabasilar acantholytic dermatitis at all levels of the epidermis

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