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Early leonine facies with alopecia in a young man

A man in his 20s presented with a 1-year history of diffuse thickening of the skin over forehead, eyebrows, cheeks, nose, and ears, leading to an appearance of swollen face with enlarged ears. Over the past 5 months, he also developed few asymptomatic skin-colored eruptions in the infra-orbital areas and alae of the nose with loss of hairs from the lateral aspects of the eyebrows. There was no family history of similar skin lesions. He was being treated for seizure disorder with sodium valproate 1400 mg once daily for the past 8 years with adequate control. On cutaneous examination, there was diffuse and waxy infiltration of the forehead, ears, cheeks, and alae of the nose. The skin appeared smooth, shiny, and wrinkle-free over these areas. Few skin-colored, waxy, discrete, and flat-topped papular lesions of size 2–3 mm were also seen over the above-mentioned sites. These papular lesions were surmounting the infiltrated area and were arranged in a linear pattern in places. There was sparsening of hair over the lateral aspect of the eyebrows as well as over the moustache and the beard region (Fig. 1a). The ears showed diffuse thickening, which was most prominent on the ear lobes (Fig. 1b). There was no well-defined area of sensory loss on pin-prick examination. The peripheral nerves were not thickened. A slitskin smear was negative for acid-fast bacilli. Urine and serum electrophoreses were negative for monoclonal proteins. Two skin biopsy specimens from forehead and ear lobe showed smalland medium-sized elongated aggregates of basaloid cells in the superficial and deep dermis. These tumor islands showed peripheral palisading of their cells and were surrounded by a stroma with increased numbers of fibroblasts. There were multiple horn cysts, many of which showed prominent calcification. Papillary mesenchymal bodies (invaginations with fibroblasts) were seen invading the tumor cell aggregates (Fig. 2). (a) (b)