A 65-Year-Old Man with Erosions and Leafy Scaling on the Scalp

Abstract

Pemphigus foliaceus is an intraepidermal autoimmune blistering disease affecting the skin. The etiopathogenesis of the disease is characterized by acantholysis and intraepidermal blisters formation, resulting from IgG autoantibodies directed against transmembrane desmosomal glycoprotein desmoglein 1. Pemphigus foliaceus usually presents between fourth and sixth decade of life and its prevalence is comparable in both sexes. Clinically, it presents as skin erosions surrounded by erythema that heal with crusting and scaling, with sparing of the mucosal surfaces. The vesicles are superficial and fragile, thus only the resultant erosions may be observed in most cases. The lesions are most commonly presented on seborrheic regions (central face, neck, chest, and upper back). The scalp is affected in in 16–65% of patients with pemphigus. In 9–15% of cases the scalp is the first location. Hair loss, observed in 5.4% of cases, is usually non-scarring, except single cases of prolonged course with staphylococcal suprainfection. Diagnosis of pemphigus foliaceus is mainly established based on clinical features and immunofluorescence tests. On direct immunofluorescence, IgG or C3 binding to the intercellular cement substance in the upper epidermis of perilesional skin is observed. Indirect immunofluorescence microscopy reveals the presence of serum autoantibodies against desmoglein 1. Treatment of pemphigus include systemic corticosteroids with/ or without immunosuppressive agent (azathioprine, mycophenolate mofetil, mycophenolic acid or dapsone) or rituximab. Topical antiseptic agents and topical corticosteroids can also be useful.KeywordsAcantholysisBullous diseaseDesmogleinHair lossImmunofluorescencePemphigus foliaceusScalp