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Effective treatment of severe thermodysregulation by oral retinoids in a patient with recessive congenital lamellar ichthyosis
- Source :
- Clinical and Experimental Dermatology. 33:578-581
- Publication Year :
- 2008
- Publisher :
- Oxford University Press (OUP), 2008.
-
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.
- Subjects :
- Adult
Male
Pathology
medicine.medical_specialty
medicine.drug_class
Hyperkeratosis
Genes, Recessive
Sweating
Dermatology
SWEAT
Retinoids
Oral administration
medicine
Humans
Retinoid
Hypohidrosis
Heat intolerance
business.industry
Ichthyosis
Lamellar ichthyosis
medicine.disease
Dyskeratosis
Treatment Outcome
medicine.symptom
business
Ichthyosis, Lamellar
Body Temperature Regulation
Subjects
Details
- ISSN :
- 13652230 and 03076938
- Volume :
- 33
- Database :
- OpenAIRE
- Journal :
- Clinical and Experimental Dermatology
- Accession number :
- edsair.doi.dedup.....b86385abe514f602ae03c7cdaf25a205