No abstract available.
Porphyrias, Hepatic*

No abstract available.
Alcoholism* ; Porphyria Cutanea Tarda* ; Porphyrias*

No abstract available.
Humans ; Interferon-beta ; Interferons ; Multiple Sclerosis ; Porphyria Cutanea Tarda ; Porphyrias

Porphyria cutanea tarda(PCT) is a photocutaneous disorder due to excessive porphyrins in the skin. We experienced 4 male patients who showed typical clinical manifestations. Histologic findings revealed subepidermal bullae with festooned derrnal papi11ae, and typical porphyrin excretion pattern of PCT was detected. One case had on 500mg daily of chloroquine with r.linical and biochemical improvement 4 months later without any adverse effects. Other 2 cases had on 125mg of chloroquine twice a week and the other one had perforrned phlebotomy.
Chloroquine ; Humans ; Male ; Phlebotomy ; Porphyria Cutanea Tarda* ; Porphyrias* ; Porphyrins ; Skin

No abstract available.
Humans ; Kidney Failure, Chronic ; Porphyria Cutanea Tarda* ; Porphyrias* ; Renal Dialysis*

No abstract available.
Leg* ; Porphyria, Acute Intermittent* ; Porphyrias

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 coIor and fluoresced pinkish under the Wood's light. Serum iron level was markedly elevated (400 microgram%). The other abnormal findings of liver function test were BSP retention (16%/45min.), 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.
Alanine Transaminase ; Alcoholics ; Aspartate Aminotransferases ; Biopsy ; Cheek ; Cicatrix ; Foot ; Hand ; Humans ; Iron ; Liver ; Liver Cirrhosis ; Liver Function Tests ; Middle Aged ; Porphyria Cutanea Tarda* ; Porphyria, Acute Intermittent ; Porphyrias* ; Skin ; Varicose Veins

Porphyria cutanea tarda (PCT) is a metabolic disorder that results in a decrease in uroporphyrinogen decarboxylase activity. It is characterized by photosensitivity, bullae formation, and skin pigmentation. There are four types of PCT: acquired, familial, toxic, and hepatoerythropoietic. Uroporphyrin levels are elevated in the urine of PCT patients. PCT can be differentiated from other porphyrias by its clinical characteristics and the porphyrin levels in the serum, erythrocytes, urine, and feces. This metabolic disorder can lead to liver dysfunction as well as histological changes such as fatty infiltration or hepatic fibrosis. PCT rarely manifests as liver cirrhosis. We report herein a case of PCT-induced liver cirrhosis that progressed to hepatic failure.
Blister ; Erythrocytes ; Feces ; Fibrosis ; Humans ; Liver ; Liver Cirrhosis ; Liver Diseases ; Liver Failure ; Porphyria Cutanea Tarda ; Porphyrias ; Skin Pigmentation ; Uroporphyrinogen Decarboxylase

Pseudoporphyria is a generic term that is used to describe photoaggravated bullous dermatoses associated with multiple iatrogenic causes, including medications and dialysis. The bullous lesions of pseudoporphyria are similar to those of porphyria cutanea tarda, both clinically and histologically, but they occur in the absence of the abnormally high levels of porphyrins which are found in true porphyrias. Treatment entails discontinuation of suspected agents and sun protection, especially against UVA wavelengths. We report a case of pseudoporphyria in a 28-year-old male who had erythematous crusted erosions, vesicles and hyperpigmented macules on his face and both forearms. A biopsy from his Lt. forearm revealed subepidermal bullaes with festooning of dermal papillae, and mild lymphocytic perivascular infiltrations. However laboratory tests for porphyrin were negative in his urine, blood and stool.
Adult ; Biopsy ; Blister ; Dialysis ; Forearm ; Humans ; Male ; Porphyria Cutanea Tarda ; Porphyrias ; Porphyrins ; Skin Diseases, Vesiculobullous ; Solar System

Adult ; Hepatitis B ; complications ; Hepatitis B virus ; Humans ; Male ; Porphyrias, Hepatic ; virology