Multiple nonmelanoma skin cancer associated with HLA DR7 in southern Australia.
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Biomedical subjects
Publications and source records attributed to D B Czarnecki.
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Presumed skin cancers were excised from 46 consecutive outpatients without antibiotic cover. A purulent wound developed in ten patients. Similar, but contaminated lesions were excised from 40 patients, but mupirocin ointment was used before surgery. Not one purulent wound developed out of 45 excisions.
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Six patients with generalized granuloma annulare were successfully treated with Dapsone. Their ages ranged from 11 to 76. There were no serious side-effects and all were able to cease the drug.
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Zinc deficiency that was diagnosed at 14 weeks of age developed in a breast-fed premature infant. There was a rapid response to zinc supplements (20 mg/day) and therapy was stopped after three weeks without recurrent disease. The maternal breast milk had a low level of zinc and this could not be corrected by oral zinc supplements.
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A patient with active, generalized morphoea was treated successfully with salazopyrine on two occasions. The first time the trunk was involved and most of the skin softened after the drug therapy was started. After salazopyrine was discontinued, morphoea developed in the thighs but recommencement of therapy resulted in complete resolution.
A 73-year-old man developed a malignant clear cell hidradenoma on the nose. Although it was treated solely with diathermy and curettage it did not recur. These rare tumours are histologically malignant but do not always behave aggressively. In some patients local growth and recurrence may occur, while in others the tumour metastasizes widely. There is no way of predicting how these tumours will behave.
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Uroporphyrinogen decarboxylase levels were measured in haemolysed whole blood or fibroblasts from 3 unrelated patients with hepatoerythropoietic porphyria (HEP) and in 4 unrelated patients with familial porphyria cutanea tarda, a condition in which the enzyme is defective. In HEP patients enzyme activities were 7% of normal in erythrocytes and 8% of normal in cultured skin fibroblasts. All the features of HEP, including the characteristic accumulation of protoporphyrin in erythrocytes, are secondary to this enzyme defect. The father of 1 HEP patient was heterozygous for the same enzyme defect. He also had uroporphyrinuria and was therefore indistinguishable from patients with subclinical familial porphyria cutanea tarda. It is suggested that patients with HEP are homozygous for the gene that causes porphyria cutanea tarda.
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In a case of hepatoerythropoietic porphyria (HEP) with unusual features, the patient had onset of photosensitivity in infancy, followed by spontaneous resolution of photosensitivity by the age of 7 years. Seven other cases of HEP have been found in the medical literature; the disease has systemic complications, mainly liver disease and anemia, and is inherited as an autosomal recessive trait. Certain clinical and biochemical features distinguish HEP from erythropoietic porphyria and erythropoietic protoporphyria, the two diseases with which HEP is often confused.