Porohyria Cutanea Tarda.
- Author:
Young Pio KIM
;
Seok Don PARK
;
Jae il SEO
- Publication Type:Original Article
- MeSH:
Basophils;
Cicatrix;
Collagen;
Dermis;
Electrocardiography;
Electrophoresis;
Fingers;
Forehead;
Gastritis;
Hand;
Hepatocytes;
Humans;
Hyperpigmentation;
Hypertrichosis;
Hypertrophy, Left Ventricular;
Liver;
Male;
Middle Aged;
Phlebotomy;
Skin;
Thorax;
Tuberculosis;
Urine Specimen Collection;
Wood
- From:Korean Journal of Dermatology
1978;16(3):251-259
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
A 59-year-old male farmer, an alcohol addict, has experienced repeated bullous skin lesions on the erythematous base over the exposed areas of tbe skin, especially on the face and hands, after sun-light exposure. The face showed hyperpigmenation, hypertrichosis and sclerodermoid changes. And also there were hypopigmented scars on the dorsum of the hands and cIaw-1ike fingers. Characteristic laboratory findings were as follows: Urine appeared portwine color and fluoresced under the Woods Iight. A 24-hour urine collection contamed 4l32ug of uroporphyrin and 3815ug of corproporphyrin. BSP retention(7% in 45 Min.) was increased, and A/G ratio(0. 8:1) was inverted. Gamma globulin(44, 9%) was markedly increased on serum electrophoresis. Roentgenograms revealed minimal tuberculosis on the chest P-A and superficial gastritis on the upper G-I series. Electrocardiogram showed left ventricular hypertrophy. Pathological findings of the specimen obtained from sclerodermoid lesion of the forehead revealed hypermelanosis in the basal cell layer and amorphous, basophilic collagen degeneration in the upper dermis, on the other hand loss of sinusoids due to swelling of hepatocytes and chomic inflammatory cell infiltrate in the interlobular septa were noticed on the specimen taken from the liver. For treatment, phlebotomy and metabolic alkalinization were trjed.
