A 57-Year-Old Woman with Crusted Erosions on the Scalp

Abstract

Pemphigus vulgaris is a form of autoimmune bullous disease affecting the skin and the mucous membranes. The etiopathogenesis of the disease is characterized by acantholysis and intraepidermal blisters formation, resulting from IgG autoantibodies directed against transmembrane desmosomal glycoprotein desmoglein 3, and in some cases desmoglein 1. Pemphigus vulgaris predominantly affects women between 50 and 60 years of age. The initial clinical presentation usually includes erosions on the mucous membranes. Skin involvement is characterized by flaccid blisters and erosions localized mainly on the face, scalp, trunk and proximal extremities. The scalp is affected in 16–65% of patients with pemphigus vulgaris. In 9–15% of cases the scalp is the first location of lesions. Clinically, pemphigus vulgaris on the scalp presents as a crusted erosions on erythematous background. Hair loss, observed in 5.4% of cases, is usually non-scarring, except single cases of prolonged course with staphylococcal suprainfection. Diagnosis of pemphigus vulgaris is mainly established based on clinical features and immunofluorescence tests. Treatment of pemphigus vulgaris include systemic corticosteroids with/or without immunosuppressive agent (azathioprine, mycophenolate mofetil, mycophenolic acid or dapsone) or rituximab. Topical antiseptic agents and topical corticosteroids can be also useful.KeywordsAcantholysisBullous diseaseDesmogleinHair lossImmunofluorescencePemphigus vulgarisScalp