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One sister and one brother (of four), aged 18 and 12 years, respectively, born of a consanguineous marriage between first cousins, presented with thickening of the palms and soles after 1 year of life, which was slowly increasing, and the simultaneous appearance of thickened skin: colored plaques on the dorsal aspect of the hands, feet, knees, and ankles. There was hyperhidrosis of the palms and soles, but no history of similar complaints in the maternal/paternal family. The pedigree was as shown in Fig. 1. On examination, the sister showed diffuse palmoplantar keratoderma extending to the dorsal surface with sharp margins. The fingers were short and mutilated, and the nails showed subungual hyperkeratosis, onycholysis, and Beau's lines (Figs 2 and 3). Circular hyperkeratotic knuckle‐pad‐like thickenings were present on all proximal interphalangeal joints and some knuckles. Irregular‐shaped hyperkeratotic plaques and linear keratosis were present on the flexor aspect of the wrists and on the dorsal aspect of the feet, ankles, and knees. The brother (12 years old) showed the same type of clinical features. The histopathology revealed hyperkeratosis, hypergranulosis, acanthosis, and chronic inflammatory dermal infiltrate. General and systemic examination of all the sisters and brothers was normal. Ophthalmologic examination was normal. Oto‐rhino‐laryngologic examination showed a high arched palate. Intelligence testing of all sisters and brothers was normal. The patients were given oral vitamin A with vitamin E and topical keratolytes. They are under follow‐up at present.

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