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Narrowband ultraviolet B phototherapy associated with improvement in Netherton syndrome
Author(s) -
Maatouk I.,
Moutran R.,
Tomb R.
Publication year - 2012
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1111/j.1365-2230.2011.04231.x
Subject(s) - dermatology , medicine , narrowband , ultraviolet a , computer science , telecommunications
Summary 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.