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KMID : 0363219730110030171
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Korean Journal of Dermatology
1973 Volume.11 No. 3 p.171 ~ p.177
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A Case of Porphyria Cutanea Tarda
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Abstract
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Porphyria is a rare metabolic disorder in this country and a few cases of acute intermittent porphyria has been reported. We observed a case of porphyria cutanea tarda associated with liver cirrhosis.
The patient was 61-year-old farmer with heavy alcoholic habit. He had been suffered from skin fragility and photosensitivity for 3 years. His face color was slate blue and sclerdermoid appearance noted especially on the cheek. Bullae, which is healing slowly and followed by atrophic pigmented scars, were developed on the dorsum of hand and feet after receiving trivial trauma and massive alcohol intake.
None of his family members has similar symptoms.
Urine specimen showed port-wine color and fluoresced pinkish under the Wood´s light. Serum iron level was markedly elevated (400 ,μg %). The other abnormal findings of liver function test were BSP retention (16%/45 min.), elevated SGOT (198 unit) and SGPT (80 unit) levels.
Esophagram revealed suspicious of varices and liver scanning showed cirrhotic changes.
Skin biopsy specimens taken from the cheek and dorsum of hand showed sclermoid changes and subepidermal bulla. Liver biopsy disclosed mild degree of cirrhotic changes.
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