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*

Porphyria cutanea tarda (PCT) is a disease characterized by an early appearance of severe photosensitivity with an increase in urinary uroporphyrin excretion and other porphyrins, high isocoproporphyrin fecal levels and an accumulation of protoporphyrin in erythrocyte. This disease has been known to be caused by a dramatic decrease in the activity of the uroporphyrinogen decarboxylase. Multiple infectious and environmental factors may contribute to PCT by influencing oxidative stress in the hepatocytes. We report here on a case of porphyria cutanea tarda that presented with jaundice and photosensitivity.
Adult ; English Abstract ; Humans ; Jaundice/*complications ; Male ; Photosensitivity Disorders/complications ; Porphyria Cutanea Tarda/complications/*diagnosis

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

BACKGROUND: Although there are varied etiological factors in the pathogenesis of psoriasis, recent data where viral hepatitis is more frequently found in psoriasis patients than in the normal population, suggests the possibility that some kinds of viral antigens may participate as causative factors of psoriasis. Hepatitis C virus(HCV) in particular, which has been proven to be associated with many dermatoses such as porphyria cutanea tarda, lichen planus and vasculitis, is currently suspected as a possible pathogen in psoriasis. OBJECTIVE: This study was conducted to estimate the prevalence of viral hepatitis infection among psoriasis patients in Korea where it is known to be endemic. Additionally, we analysed the relationship between the clinical severity of psoriasis and viral hepatitis according to the serotypes. METHODS: In this study, we looked for the presence of HCV, HBV, HAV, HDV and HEV infection in 65 enrolled psoriasis patients and 98 non-psoriatic patients by enzyme immunoasay, recombinant immunoblotting, radioimmunoassay and reverse transcription polymerase chain reactions. RESULTS: 1. We found a higher prevalence of viral hepatitis in the psoriasis group(70.8%) than the control group(45.9%). 2. 10.7% of patients were shown to be infected with HCV in the psoriasis group, in comparison with 2% in the control group. Also 52.7% of psoriatic patients were positive to HBV markers as HBcAb and HBsAg in comparison with 38.8% in the control group. 3. There was a statistically significant correlative relationship between the clinical severity of psoriasis and the presence of viral hepatitis. 4. We could establish a significant association among higher titers of ALT, positivity of HBcAb and HBsAg, and HCV hepatitis in psoriatic patients. CONCLUSION: Psoriatic patients had a higher prevalence of viral hepatitis than normal controls. Also psoriatic patients with viral hepatitis showed relatively different clinical features and labaratory results in comparison with those without it.
Antigens, Viral ; Hepatitis B Surface Antigens ; Hepatitis C ; Hepatitis* ; Humans ; Immunoblotting ; Korea ; Lichen Planus ; Polymerase Chain Reaction ; Porphyria Cutanea Tarda ; Prevalence ; Psoriasis* ; Radioimmunoassay ; Reverse Transcription ; Skin Diseases ; Vasculitis

Chronic HCV infection has been reported to be associated with several extrahepatic conditions such as cryoglobulinemia, lymphoma, lichen planus, porphyria cutanea tarda, autoimmune thyroiditis, and renal disease. Glomerular disease may occur in patients with chronic HCV infection. The most common patterns are membranoproliferative glomerulonephritis, and less frequently, membranous nephropathy, fibrillary glomerulonephritis and immunotactoid glomerulopathy. Few crescentic glomerulonephritis has been reported in association with HCV infection, and no case was reported in Korea yet. We experienced one case of rapidly progressive glomeruloinephritis with the pathology of crescentic glomerulonephritis complicated in membranoproliferative glomerulonephritis in a patient with HCV infection.
Cryoglobulinemia ; Glomerulonephritis* ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Hepacivirus* ; Hepatitis C* ; Hepatitis* ; Humans ; Korea ; Lichen Planus ; Lymphoma ; Pathology ; Porphyria Cutanea Tarda ; Thyroiditis, Autoimmune

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