豪猪状鱼鳞病一例诊断与治疗观察

Diagnosis and treatment of porcupine-like ichthyosis： a case report

患儿男,10岁.因全身出现红色角化性扁平丘疹及褐黄色钉状突起性角化性斑块来我所就诊.皮肤科检查：头面部、躯干、四肢大片红色角化斑块基础上疣状、钉样突起,表面结褐黄色油腻痂,皮损多融合,如＂豪猪＂样外观.背部皮损组织病理学检查示角化过度,局部柱状角化不全,棘层增生肥厚,角质层偶见棘突松解性角化不良细胞,真皮乳头不规则向上增生,浅层血管周围淋巴组织细胞浸润,根据病理结果诊断为豪猪状鱼鳞病.给予口服阿维A胶囊及外用0.1%的维A酸乳膏持续治疗8年,起始剂量0.5mg·kg-1·d-1,最大剂量1 mg·kg-1·d-1.治疗期间定期监测患儿肝肾功能,血脂及身高、体质量,不良反应除皮肤黏膜干燥外,其他无异常.

A 10-year-old boy presented with a 3-year history of erythematous flat keratotic papules and brown-yellow, nail-like prominent keratotic plaques all over the body surface. Dermatological examination showed verrucous or nail-like prominence over multiple erythematous keratotic plaques on the head, face,trunk and limbs. The lesions, most of which confluenced, were covered with brown-yellow and greasy crusts,and gave a porcupine-like appearance. Skin biopsy of lesions from the back revealed epidermal hyperkeratosis,focal columnar parakeratosis, acanthosis, few acantholytic and dyskeratotic cells in stratum corneum, irregular upward proliferation of dermal papilla cells, and a superficial perivascular lymphocytic infiltration. A diagnosis of ichthyosis hystrix was established based on the histopathological findings. The boy was treated with oral acitretin and topical 0.1% acitretin cream for 8 years. The initial and maximum dose of oral acitretin was 0.5 mg·kg-1·d-1 and 1 mg·kg-1·d-1, respectively. Liver and kidney function, body height and weight were examined during the treatment, and no side effect was observed except for skin xerosis.