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A Case of Waxy Keratoses of Childhood

Waxy keratosis of childhood was first described by Coleman et al. in 1994 [1] . Happle et al. [2] suggested the new term ‘kerinokeratosis papulosa’ for this entity in 2004. To our knowledge, 5 cases with waxy keratoses of childhood have been reported to date. We present the sixth one. A 16-year-old white boy presented with multiple small papules on his arms and legs. They had developed 8 years before and gradually increased. The lesions were asymptomatic and otherwise he was healthy. His family history for skin disorders was negative. Numerous, flesh-colored and brownish, flat or hyperkeratotic papules varying in size from pin head to 7 mm diameter were observed predominantly on the legs, and there were fewer lesions on the arms. Some of them were grouped on the lateral parts of the thighs and the others were discrete ( fig. 1 ). The lesions were not follicular and were painlessly detachable. The examination of the nails and mucosal membranes revealed no abnormalities. A 4-mm punch biopsy was performed and histopathological examination revealed lamellar and compact hyperkeratosis, papillomatosis with tenting of the epidermis and a pronounced stratum granulosum ( fig. 2 ). Waxy keratosis of childhood was diagnosed clinically and histopathologically. Waxy keratosis of childhood presents as well-demarcated keratotic papules secondary to abnormal keratinization. The lesions are usually localized on the trunk and proximal limbs [1, 2] . Mehrabi et al. [3] reported a case of waxy keratoses of childhood with segmental distribution, and Happle et al. [2] described a pronounced segmental involvement superimposed on mild, nonsegmental lesions of the ordinary type. Our case did not have any lesions over the trunk; the lesions were predominantly localized on the thighs. Previously, 5 cases with waxy keratoses of childhood were reported. All of these were female and developed lesions before the age of 4 years. However, our patient was male and the lesions appeared when he was 8 years old. Two of the previously reported subjects were siblings, whereas the others did not have any family history, like our case [1–3] . Waxy keratosis of childhood is a new skin disorder and only few incidences have been reported. The disease has not been defined exactly yet and it is still unclear whether it is a familial disorder or whether it is only seen in childhood. We suggest that waxy keratosis of childhood should be considered in cases presenting with small hyperkeratotic papules. Future reports will provide more information about the details of this disorder. Published online: May 27, 2008