Normal anagen effluvium as the presenting sign of pemphigus vulgaris: a case report

Abstract

Pemphigus vulgaris is an autoimmune blistering diseases of skin and mucous membranes characterized histologically by intraepidermal blisters and immunopathologically by the bound and circulating IgG directed to Dsg3 and, in part, Dsg1 of the epithelial desmosomes. In majority of patients, painful mucous membrane erosions, especially in oral cavity, are the presenting sign, and scalp involvement as a initial presentation is reported to occupy 3–6% in pemphigus vulgaris. Hair follicle is one of the preferential targets for pemphigus autoantibodies as the desmosomal proteins are overexpressed in follicular epithelium. Those proteins are especially distributed throughout the outer root sheath of the whole hair follicle and in the dermal bulb matrix cell and take charge of attachment of hair. A 47-year-old Korean man presented with crusted non-scarring alopecic patches on the scalp for 1 month and small blisters on the trunk and upper extremities for 1 week. Hairs were easily plucked from perilesional area and plucked hairs looked like normal anagen hairs with intact root sheaths. Histopathologic examination revealed acantholysis of basal layer in epidermis by the manner of “row of tombstones” and clefts in outer root sheathes of hair follicles. Direct immunifluorescence test, indirect immunofluorescence test and immunoblotting test were consistent with pemphigus vulgaris. This case shows normal anagen effluvium as well as painful oral mucous membrane erosions can be a presenting signs of pemphigus vulgaris.