delta-Aminolaevulinate dehydratase activity was measured in liver biopsy specimens from 13 patients with porphyria cutanea tarda, 13 alcoholics and 10 control subjects. The mean enzyme activities in liver of porphyria cutanea tarda and alcoholics were 34% (P less than 0.001) and 49% (P less than 0.001) respectively of the control level. Heat treatment of liver supernatant caused slight inhibiti...

Porphyria cutanea tarda has a complex etiology with genetic factors not completely elucidated. The miscegenation of the Brazilian population has important implications in the predisposition to diseases. There are no studies concerning the genetic ancestry of patients with porphyria cutanea tarda from a mixed population. Thirty patients living in Rio de Janeiro with sporadic porphyria cutanea ta...

The manifestation of porphyria cutanea tarda reflects genetic and environmental factors. Mutations in the uroporphyrinogen decarboxylase gene, located at chromosome 1p34, discriminate familial porphyria cutanea tarda from sporadic cases. Furthermore, mutations in the haemochromatosis gene may be involved in the aetiology. In this study 53 unrelated Danish patients with porphyria cutanea tarda w...

Porphyria cutanea tarda is prevalent in connective tissue disease, common in systemic lupus erythematosus. However, the co-existence of primary sjogren's syndrome and porphyria cutanea tarda is rare and poses diagnostic and therapeutic challenges. We report a case of porphyria cutanea tarda associated with primary sjogren's syndrome.

In many countries hepatitis C virus infection has been considered a major factor triggering overt porphyria cutanea tarda. The prevalence of hepatitis C virus infection was retrospectively studied in 87 patients who during a period of 11 years were diagnosed with porphyria cutanea tarda in Stockholm. Among patients with the sporadic form of porphyria cutanea tarda, the prevalence of hepatitis C...

BACKGROUND Porphyria cutanea tarda is the most common form of porphyria, characterized by the decreased activity of the uroporphyrinogen decarboxylase enzyme. Several reports associated HFE gene mutations of hereditary hemochromatosis with porphyria cutanea tarda worldwide, although up to date only one study has been conducted in Brazil. OBJECTIVES Investigation of porphyria cutanea tarda ass...

1. Erythrocyte uroporphyrinogen decarboxylase activity has been measured in 27 patients with porphyria cutanea tarda, of whom 11 had a family history of overt porphyria cutanea tarda. 2. Eight patients from six families had erythrocyte uroporphyrinogen decarboxylase activities that were decreased to about half of control values. This decrease was shown by family studies to be inherited as an au...

Pseudoporphyria is an uncommon bullous disease with similar clinical and histopathological findings to porphyria cutanea tarda, in the absence of detectable porphyrin elevation. We present a 34-year-old man with clinical and histological findings compatible with porphyria cutanea tarda, whose his urinary uroporphyrin was negative and we concluded that he was a case of pseudoporphyria. Concomita...

Uroporphyrinogen decarboxylase activity was measured in liver and erythrocytes of normal subjects and in patients with porphyria cutanea tarda and their relatives. In patients with porphyria cutanea tarda, hepatic uroporphyrinogen decarboxylase activity was significantly reduced (mean 0.43 U/mg protein; range 0.25-0.99) as compared to normal subjects (mean 1.61 U/mg protein; range 1.27-2.42). E...

This is a case report about Porphyria cutanea tarda (PCT) and its relationship with the infection caused by the human immunodeficiency virus (HIV). Cutaneous porphyria is an illness caused by enzymatic modification that results in partial deficiency of uroporphyrinogen decarboxylase (Urod), which may be hereditary or acquired. Several studies suggest that HIV infection associated with cofactors...