Chaves Antonio J, Merchán-García R, Fernández-Recio José Maria, Rodríguez-Nevado Isabel, de Argila Diego
Unidad de Dermatología, Hospital Universitario Infanta Cristina, Badajoz, España.
Actas Dermosifiliogr. 2006 Jun;97(5):342-4. doi: 10.1016/s0001-7310(06)73415-9.
We describe the case of a 32-year-old male patient who had presented from birth with generalized ichthyosiform dermatosis, palmoplantar keratoderma with constrictive bands around the fingers and keratotic plaques in a linear arrangement, located in the large skin folds. The dermatopathological examination showed orthokeratotic hyperkeratosis and epidermal hyperplasia with hypergranulosis. No other members of the patient's family were affected. With these clinical and histological findings, the diagnosis was keratosis linearis with ichthyosis congenita and sclerosing keratoderma (KLICK syndrome). The patient rejected treatment with oral retinoids and was treated with emollients and 30 % urea creams, with little clinical response.
我们描述了一名32岁男性患者的病例，该患者自出生以来就患有全身性鱼鳞病样皮肤病、掌跖角化病，手指周围有紧缩带，以及呈线性排列的角化性斑块，位于大的皮肤褶皱处。皮肤病理检查显示正角化过度角化和伴有颗粒层增厚的表皮增生。患者家族中无其他成员患病。根据这些临床和组织学表现，诊断为先天性鱼鳞病和硬化性角化病伴线状角化病（KLICK综合征）。患者拒绝口服维甲酸治疗，接受了润肤剂和30%尿素乳膏治疗，但临床反应甚微。
