Abstract |
Netherton syndrome (NS) is a rare and severe autosomal recessive ichthyosis. We report the case of a patient with NS treated successfully with narrowband ultraviolet B (NB-UVB) phototherapy after failure of low-dose oral isotretinoin. A 16-year-old girl born to consanguineous parents presented with severe ichthyosis. On examination of her hair, she was found to have trichorrhexis invaginata. Genetic analysis revealed a SPINK5 mutation specific for NS. The patient's cousin had the same mutation. The patient developed Cushing syndrome after long-term application of topical steroids. She was treated with low-dose oral isotretinoin with no benefit. NB-UVB phototherapy was started, which resulted in marked improvement after 2 months. In conclusion, we report a case of NS with failure of low-dose oral isotretinoin, for which NB-UVB phototherapy, through its apoptosis-inducing and immunomodulatory effects, was effective. However, long-term use of UVB is limited by its potential side-effects.
|
Authors | I Maatouk, R Moutran, R Tomb |
Journal | Clinical and experimental dermatology
(Clin Exp Dermatol)
Vol. 37
Issue 4
Pg. 364-6
(Jun 2012)
ISSN: 1365-2230 [Electronic] England |
PMID | 22582910
(Publication Type: Case Reports, Journal Article)
|
Copyright | © The Author(s). CED © 2012 British Association of Dermatologists. |
Chemical References |
- Dermatologic Agents
- Isotretinoin
|
Topics |
- Adolescent
- Dermatologic Agents
(therapeutic use)
- Female
- Humans
- Isotretinoin
(therapeutic use)
- Netherton Syndrome
(therapy)
- Treatment Outcome
- Ultraviolet Therapy
(methods)
|