School in photodermatology: Smith-Lemli-Opitz syndrome.
The Smith-Lemli-Opitz syndrome has only recently been added to the small number of congenital disorders characterized by photosensitivity. The clinical features of this disorder are distinct from other photosensitivity syndromes. Details on the patho-mechanism of photosensitivity in the Smith-Lemli-Opitz syndrome have yet to be fully determined. However, preliminary evidence points to the deranged cholesterol metabolism that characterizes the syndrome as causal in this UVA-mediated photosensitivity disorder.
Anstey
Alex
A
Academic Department of Dermatology, College of Medicine, Cardiff University, Cardiff, UK. alex.anstey@gwent.wales.nhs.uk
D006801:Humans; D050356:Lipid Metabolism; D010787:Photosensitivity Disorders / Q000209:etiology*; D011481:Protective Clothing; D019082:Smith-Lemli-Opitz Syndrome / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism* / Q000473:pathology / Q000628:therapy; D013473:Sunscreening Agents / Q000627:therapeutic use; D013577:Syndrome; D014466:Ultraviolet Rays / Q000009:adverse effects
D013473:Sunscreening Agents