Haenssle H A, Finkenrath A, Hausser I, Oji V, Traupe H, Hennies H C, Neumann C, Emmert S
Department of Dermatology, Laboratory for Electron Microscopy, University of Heidelberg, Heidelberg, Gemany.
Clin Exp Dermatol. 2008 Aug;33(5):578-81. doi: 10.1111/j.1365-2230.2008.02709.x. Epub 2008 Mar 18.
Ichthyoses are a heterogenous group of keratinization disorders, which are often associated with hypohidrosis. We report a 42-year-old man with generalized thick brownish scales and severe thermodysregulation leading to heat intolerance. Based on clinical, histological and genetic findings, autosomal recessive lamellar ichthyosis (ultrastructural electron microscopy type III) was diagnosed. Severe generalized hypohidrosis both at rest and after physical exercise (bicycle ergometer) was documented. Oral treatment with retinoids not only markedly improved the skin condition but also restarted the ability of the patient to thermoregulate by perspiration. The normalization of sweat-gland function was confirmed by gravimetrically measuring sweat rates before and after retinoid treatment. This report shows that retinoid therapy can markedly improve the quality of life in patients with generalized lamellar ichthyosis by reconstitution of perspiration.
鱼鳞病是一组异质性角化障碍性疾病，常伴有少汗症。我们报告了一名42岁男性，其全身有厚的褐色鳞屑，伴有严重的体温调节障碍，导致不耐热。根据临床、组织学和遗传学检查结果，诊断为常染色体隐性遗传性板层状鱼鳞病（超微结构电子显微镜III型）。记录显示患者在静息状态和体育锻炼（自行车测力计）后均有严重的全身少汗症。口服维甲酸治疗不仅显著改善了皮肤状况，还恢复了患者通过出汗进行体温调节的能力。通过重量法测量维甲酸治疗前后的出汗率，证实了汗腺功能的恢复正常。本报告表明，维甲酸治疗可通过恢复排汗功能显著改善泛发性板层状鱼鳞病患者的生活质量。
