Effective treatment of severe thermodysregulation by oral retinoids in a patient with recessive congenital lamellar ichthyosis

Haenssle, H.A., Finkenrath, A., Hausser, I., Oji, V., Traupe, H., Hennies, Hans C., Neumann, C. and Emmert, S. (2008) Effective treatment of severe thermodysregulation by oral retinoids in a patient with recessive congenital lamellar ichthyosis. Clinical and Experimental Dermatology, 33 (5). pp. 578-581. ISSN 0307-6938

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Abstract

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.

Item Type:	Article
Additional Information:	Unmapped bibliographic data: ST - Effective treatment of severe thermodysregulation by oral retinoids in a patient with recessive congenital lamellar ichthyosis [Field not mapped to EPrints] LB - 3 [Field not mapped to EPrints] RP - Not in File [Field not mapped to EPrints]
Subjects:	Q Science > QH Natural history > QH301 Biology Q Science > QH Natural history > QH426 Genetics
Schools:	School of Applied Sciences
Related URLs:	Author
Depositing User:	Hans Hennies
Date Deposited:	13 Jul 2016 15:38
Last Modified:	01 Apr 2018 18:15
URI:	http://eprints.hud.ac.uk/id/eprint/28803

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