title: Narrowband ultraviolet B phototherapy associated with improvement in Netherton syndrome.
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.affiliation: Department of Dermatology, Hôtel-Dieu de France, Ashrafieh, Beirut, Lebanon. ismaelmaatouk@yahoo.com
authors.forename: I
authors.initials: I
authors.lastname: Maatouk
authors.forename: R
authors.initials: R
authors.lastname: Moutran
authors.forename: R
authors.initials: R
authors.lastname: Tomb
mesh_terms: D000293:Adolescent; D003879:Dermatologic Agents / Q000627:therapeutic use; D005260:Female; D006801:Humans; D015474:Isotretinoin / Q000627:therapeutic use; D056770:Netherton Syndrome / Q000628:therapy*; D016896:Treatment Outcome; D014467:Ultraviolet Therapy / Q000379:methods*
chemical_list: D003879:Dermatologic Agents; D015474:Isotretinoin