A 4-year-old Caucasian boy with non-consanguineous parents was admitted to the Department of Dermatology for evaluation of asymptomatic keratotic papules on the dorsal aspects of both hands (Fig. 1a). The lesions had developed 2 years prior to hospitalization and had gradually increased in number. Treatment with topical corticosteroids had been ineffective. There was no family history of similar lesions and the boy was otherwise healthy. No abnormalities of the hair, nail or teeth were seen on physical examination. Full blood count, routine biochemical analysis and urinalysis were within normal limits. A 4-mm punch biopsy was performed from lesional skin (Fig. 1b). Orcein staining revealed normal elastic fibres.