Posts Tagged “ porphyria ”

Written by Gabriela Segura, MD
Saturday, 7 February 2009 13:21

There are several skin diseases that are exacerbated and/or precipitated with sunlight – lupus, rosacea, pellagra, and porphyria among others- from which a specific subtype of porphyria is the basis of the Werewolf Legend. I’m talking here about Congenital Erythropoietic Porphyria or Günther’s disease, a very rare (less than 100-200 cases) and severe form of porphyria where there is a lack of a specific enzyme in the bone marrow, urogen III synthase also known as cosynthetase. Children with this condition typically are hairy, they have red urines, and they have extreme sensitivity to sunlight which leaves multiple scars and mutilations. Treatment is based in protection from the sun and splenectomy. Another form of porphyria, Porphyria Cutanea Tarda, is also characterized by sensitivity to the sun with subsequent overgrowth of hair in the face plus other skin erosions like blisters and others.

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