Successful Treatment of Localized Pemphigus Foliaceus with Topical Pimecrolimus

We report the case of successful treatment of a 79-year-old male patient with recurrent pemphigus foliaceus with pimecrolimus cream 1% once daily for 40 days. The patient initially presented with localized lesions on the scalp and nose area and was treated with systemic corticosteroids. At his fourth relapse within a period of 16 months, he refused any systemic treatment. Pimecrolimus cream was suggested to him as an alternative option. 1. Introduction Pemphigus foliaceus (PF) is a chronic autoimmune blistering disease mainly affecting the cornified skin of the face and upper torso, such as the presternal and interscapular regions, rather than the lower torso or the scalp. PF, as well as pemphigus vulgaris (PV), is characterized by the loss of subcorneal keratinocyte cell adhesion. The latter is clinically expressed by the formation of fragile vesicles which rupture easily, leaving behind erosions. In PF, pathogenic immunoglobulin G (IgG) targets the desmosome cadherin desmoglein1, a 160？KDa, calcium-dependent, transmembrane glycoprotein that plays an important role in cell-to-cell adhesion of the most differentiated epidermal epithelia. Most cases of PF are treated with systemic glucocorticosteroids with or without immunosuppressive therapy, although some mild cases can respond well to topical glucocorticosteroids alone [1]. We report a case of PF with recurrent localized lesions at the face and scalp area, which was successfully treated with topical pimecrolimus. 2. Case Report We reviewed a 79-year-old patient in the outpatient department of our hospital. He presented with scalp erosions which had been covered with markedly hyperkeratotic scaling for the last 4 months (Figure 1(a)). He also reported mild pruritus. No previous trauma, surgery, irradiation, or any topical treatment preceded the appearance of these lesions. The lesions had been treated unsuccessfully in the past with cryotherapy. From his previous medical history, he reported rosacea of the cheeks under treatment. Figure 1: (a) Markedly hyperkeratotic scaling and erosions on scalp. (b) Subcorneal vesicle with a small number of dyskeratotic granular cells (H&E ×40). (c) Erosions and hyperkeratotic, cobblestone-like crusting on the nose of the same patient. (d) Clearance of lesions six months after the end of the pimecrolimus course. A skin biopsy from the scalp was performed (Figure 1(a)). The histology report revealed features of pemphigus foliaceus, such as formation of superficial bullae with acantholytic cells, parakeratosis, acanthosis, and slight spongiosis (Figure 1(b)). The