Porphyria cutanea tarda
+Ufimtseva M.A., Kaneva E.V., Khudorozhkova N.P.

Ural State Medical University, Ekaterinburg, Russia

Porphyria cutanea tarda is the most prevalent subtype of porphyria and the most common inherited condition produced by additional exogenous factors. The paper reviews etiology and pathogenesis as well as clinical signs of porphyria cutanea tarda. Currently, this subtype of porphyria is considered as a multifactorial disorder which is resulted from the latent or manifest liver damage followed by porphyrin metabolism abnormalities. A variety of exogenous and endogenous factors contribute to the pathogenesis of this disease. The paper addresses the classification of clinical manifestations of porphyria cutanea tarda. It is classified by the prevalent site of lesion into cutaneous, cutaneous visceral, cutaneous nervous, and mixed subsets. 80% of these patients present with atypical forms, i.e., scleroderma-like, ulcero-necrotic, or melanoderma-like. Inherited erythropoietic and hepatic porphyrias are diagnosed by typical abnormalities in laboratory tests and skin manifestations. Major diagnostic criteria of porphyria cutanea tarda and pathognomonic symptoms are discussed. The clinical case illustrates the diagnosis of porphyria cutanea tarda in an HIV-positive woman.

Key words: HIV-positive patient, multifactorial disorder, HIV infection, porphyrins.

For citation: Ufimtseva M.A., Kaneva E.V., Khudorozhkova N.P. Porphyria cutanea tarda // RMJ. Dermatology. 2016. № 10. P. 651–654.