Subject(s)
Liver/pathology , Porphyrias/pathology , Skin Diseases/pathology , Adult , Aged , Alcohol Drinking , Biopsy , Chloroquine/therapeutic use , Ethiopia , Female , Humans , Inflammation/pathology , Liver Function Tests , Male , Middle Aged , Porphyrias/drug therapy , Porphyrias/epidemiology , Skin Diseases/drug therapy , Skin Diseases/epidemiology , Uroporphyrins/urineSubject(s)
Glomerulonephritis/diagnosis , Nephrotic Syndrome/diagnosis , Adolescent , Adult , Ethiopia , Female , Humans , Male , Middle Aged , Nephrotic Syndrome/etiologySubject(s)
Glomerulonephritis/pathology , Adolescent , Adult , Female , Glomerulonephritis/drug therapy , Humans , Kidney/drug effects , Kidney/pathology , Male , Middle Aged , Prognosis , Prospective StudiesABSTRACT
Porphyria cutanea tarda (PCT) is common in Ethiopia and invariably affects the liver. Treatment by abstension from alcohol and avoidance of direct sunlight often failed to achieve lasting improvement. Phlebotomy is unacceptable to most of our patients and impractical as a routine therapy. Chloroquine phosphate 500 mg (300 mg base) given daily for 10 days to 24 patients with confirmed PCT, was found to be uniformly effective. Both clinical and biochemical remissions were complete, The side effects of chloroquine include fever, nausea, vomiting and myalgia which predictably occur on the third day of therapy and subside within 72 hours. Since all other modes of therapy are ineffective or impractical and since the response to chloroquine is prompt, effective and reproducible and the side effects are brief, mild and do not cause permanent hepatic damage, it is suggested that this drug is currently the most practical treatment for PCT in areas where repeated phlebotomy is unacceptable and patient follow-up is unsatisfactory.
Subject(s)
Chloroquine/therapeutic use , Porphyrias/drug therapy , Skin Diseases/drug therapy , Adult , Aged , Female , Humans , Male , Middle Aged , Porphyrias/metabolism , Porphyrins/metabolism , Skin Diseases/metabolismABSTRACT
The clinical, biochemical and histological features of 75 Ethiopians with porphyria cutanea tarda (PCT) are described. PCT in Ethiopia is definitely related to alcohol abuse and there is no clinical evidence for hereditary predisposition. Significant elevation of transaminases and bromsulphthalein retention, moderate to marked hepatic siderosis and inflammation with little or no fibrosis, suggestive of mild or likely reversible parenchymal changes, characterize the liver affection in these patients. A similar study of 18 patients with hyperpigmentation of the face and hands but without blisters, an unexplained but common feature of liver disease in Ethiopia, revealed that serum iron and urinary uroporphyrin levels were normal in eight but significantly elevated in ten (56%). Neither elevated serum iron and hepatic siderosis nor increased urinary uroporphyrin completely explains the hyperpigmentation in this group of patients.
Subject(s)
Porphyrias/metabolism , Skin Diseases/metabolism , Adult , Aged , Alcohol Drinking , Ethiopia , Female , Humans , Liver Diseases/metabolism , Liver Function Tests , Male , Middle Aged , Pigmentation Disorders/metabolism , Porphyrias/etiology , Porphyrins/urine , Prospective Studies , Skin Diseases/etiology , SunlightABSTRACT
Hyperpigmentation of the face and hands without blisters and hypertrichosis in Ethiopian patients with liver disease represents an early or latent form of porphyria cutanea tarda.
Subject(s)
Facial Dermatoses/diagnosis , Hand Dermatoses/diagnosis , Pigmentation Disorders/diagnosis , Porphyrias/diagnosis , Ethiopia , Facial Dermatoses/etiology , Female , Hand Dermatoses/etiology , Humans , Liver Diseases/complications , Male , Pigmentation Disorders/etiology , Porphyrias/etiology , Time FactorsABSTRACT
The results are reported of a prospective study of liver histological findings in twenty-five patients with severe psoriasis subjected to liver biopsy before and at intervals after the onset of treatment with methotrexate by an intermittent dosage schedule. The pre-methotrexate liver biopsy findings in forty-one other severe psoriatics are also summarized. Of the twenty-five treated patients, four showed a mild inflammatory cell infiltrate and two mild fibrosis before treatment. During treatment only one developed a grade 2 infiltrate and one other grade 2 fibrosis. None has developed cirrhosis. Where minor abnormalities developed, their fluctuating and reversible nature is demonstrated.
