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      Internal disorders associated with bullous disease of the skin

      Journal of the American Academy of Dermatology
      Elsevier BV

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          Decreased activity of hepatic uroporphyrinogen decarboxylase in sporadic porphyria cutanea tarda.

          To investigate the role of uroporphyrinogen decarboxylase in the pathogenesis of the sporadic form of porphyria cutanea tarda, we measured this enzyme in liver, erythrocytes and cultured skin fibroblasts, and also measured coproporphyrinogen oxidase and the total iron concentration in liver. The mean uroporphyrinogen decarboxylase activity was lower in liver from seven male patients (9.0 pmol of coproporphyrin per minute per milligram of protein) than in 12 controls, including seven with alcoholic liver disease (22.3 pmol per minute per milligram; P less than 0.05). Coproporphyrinogen oxidase activities were the same in each group. Liver iron concentrations were lower during remission, but uroporphyrinogen decarboxylase activities were not related to clinical activity for uroporphyrin excretion. Erythrocyte and fibroblast enzyme activities were the same as in normal subjects. A hepatic uroporphyrinogen decarboxylase defect is a prerequisite for the development of porphyria cutanea tarda, but other factors, which probably do not alter uroporphyrinogen decarboxylase activity, determine the clinical onset. In sporadic porphyria cutaneous tarda, the enzyme defect appears to be restricted to the liver.
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            Pemphigus: a 20-year review of 107 patients treated with corticosteroids.

            We review 107 consecutive cases of pemphigus. The mortality for pemphigus vulgaris and pemphigus vegetans was 46% between 1949 and 1959 and 24% between 1960 and 1970. The overall mortality for all types of pemphigus was 32%. In the corticosteroid era, complications of therapy were the most frequent causes of death. Mortality and morbidity closely correlated with the corticosteroid dosage used to attain control. This dosage proved to be variable and could not be predicted at the outset in any given patient.
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              Immunopathological investigations in the Senear-Usher syndrome (coexistence of pemphigus and lupus erythematosus).

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                Author and article information

                Journal
                Journal of the American Academy of Dermatology
                Journal of the American Academy of Dermatology
                Elsevier BV
                01909622
                August 1980
                August 1980
                : 3
                : 2
                : 107-119
                Article
                10.1016/S0190-9622(80)80247-7
                952e1755-02a1-4e43-848c-1f8c1f7d4f64
                © 1980

                http://www.elsevier.com/tdm/userlicense/1.0/

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