Abstract
Scalp is a unique location for pemphigus because of the abundance of desmogleins localized in hair follicles. Scalp involvement is observed in up to 60% of patients in the course of pemphigus. The lesions may occasionally lead to alopecia. Unforced removal of anagen hairs in a pull test is a sign of high disease activity. Direct immunofluorescence of plucked hair bulbs is considered a reliable diagnostic method in patients with pemphigus. Follicular acantholysis is a characteristic histopathological feature of pemphigus lesions localized on the scalp. Trichoscopy may serve as a supplementary method in the diagnosis of pemphigus. This review summarizes the most recent data concerning scalp involvement in pemphigus vulgaris and pemphigus foliaceus. A systematic literature search was conducted in three medical databases: PubMed, Embase, and Web of Science. The analysis included literature data about desmoglein distribution in hair follicles, as well as information about clinical manifestations, histopathology, immunopathology, and trichoscopy of scalp lesions in pemphigus and their response to treatment.
Highlights
Pemphigus is a group of autoimmune bullous diseases, in which anti-desmoglein autoantibodies induce the loss of keratinocyte adhesion, leading to blister formation
Hypotrichosis with microscopically normal hairs was observed in a mutation of Desmoglein 1 (Dsg1) gene causing SAM syndrome [8]
Some authors suggest that desmoglein 4 may play a role as an additional autoantigen in pemphigus vulgaris [9]
Summary
Pemphigus is a group of autoimmune bullous diseases, in which anti-desmoglein autoantibodies induce the loss of keratinocyte adhesion, leading to blister formation. Anti-desmoglein 1 autoantibodies may be detected in more than half of patients with pemphigus vulgaris [1]. Desmoglein 1 is the main autoantigen of pemphigus foliaceus. The significance of the distribution of desmogleins in hair follicles for scalp involvement [6] and a potential use of direct immunofluorescence of plucked hairs are discussed in the literature [7]. The significance of scalp involvement for the course of pemphigus remains controversial. The aim of this systematic review was to collect and critically analyze data concerning the types of alopecia occurring in pemphigus, the usability of direct immunofluorescence of plucked hairs as well as trichoscopy in diagnosing pemphigus, the histopathological picture of scalp involvement in pemphigus, and the responsiveness to treatment in patients with scalp involvement in the course of pemphigus
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