Cases reported "Porphyria Cutanea Tarda"

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1/23. porphyria cutanea tarda. Don't forget to look at the urine.

    diagnosis of porphyria cutanea tarda is usually fairly straightforward because of the characteristic clinical findings. Blisters and erosions develop acutely on sun-exposed skin, sometimes accompanied by hypertrichosis, abnormal pigmentation, and milia formation. Iron stores are usually elevated, and liver transaminases and blood glucose levels are often above normal as well. Gross examination of the urine can provide a valuable clue, since urine of porphyria cutanea tarda patients is red to brown in natural light and pink to red in fluorescent light. biopsy of a bullous lesion is useful to rule out other diseases. Confirmation of porphyria cutanea tarda requires measurement of porphyrin levels in a 24-hour urine collection. Once the diagnosis is confirmed, it appears reasonable to screen all patients with porphyria cutanea tarda for hepatitis c and possibly B, but especially those less than 30 years old who have extremely high liver transaminase levels. Therapeutic measures for porphyria cutanea tarda include avoidance of exacerbating factors, especially ultraviolet light, ethanol, and certain medications. phlebotomy or chloroquine therapy is reserved for patients in whom conservative measures fail.
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ranking = 1
keywords = hepatitis
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2/23. Highly active antiretroviral therapy leading to resolution of porphyria cutanea tarda in a patient with AIDS and hepatitis c.

    The association between hiv infection and porphyria cutanea tarda (PCT) is not well established. Since almost all hiv-infected patients with PCT previously described in the literature had additional risk factors for PCT, it is still unclear if hiv infection and not a cofactor such as hepatitis c virus is the trigger for PCT in this population. We describe a patient with AIDS and hepatitis c who developed bullous lesions due to PCT. The cutaneous lesions persisted for 18 months and resolved after he was placed on highly active antiretroviral therapy for hiv. No other therapeutic interventions were undertaken, while exposure to other known precipitants remained unchanged. During follow-up, skin lesions reappeared when the patient discontinued antiretroviral therapy, but PCT lesions again resolved after he restarted highly active antiretroviral therapy and hiv infection was controlled. This case supports the hypothesis that a direct causative relationship exists between hiv and the development of PCT.
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ranking = 6
keywords = hepatitis
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3/23. porphyria cutanea tarda occurring in a patient with renal failure, systemic lupus erythematosus and chronic hepatitis c infection treated with hemodialysis.

    The diagnosis and management of porphyria cutanea tarda (PCT) is complicated when it occurs in the context of renal failure, chronic hemodialysis, and anemia. We report a case of a woman who presented with painful acral blisters and hyperpigmentation. Her medical history included systemic lupus erythematosus, chronic hepatitis c infection, and renal failure necessitating chronic hemodialysis with a baseline anemia. A highly elevated serum porphyrin level led to the diagnosis of PCT. Treatment with small repeated phlebotomies and concomitant administration of erythropoietin was effective in managing her PCT.
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ranking = 5
keywords = hepatitis
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4/23. Ineffective interferon treatment of chronic hepatitis c-associated porphyria cutanea tarda, but with a transient decrease in HCV rna levels.

    Many patients with porphyria cutanea tarda (PCT) have been reported to be hepatitis c virus (HCV) carriers, suggesting that HCV infection plays a role in the pathogenesis of this type of porphyria. In this study, we report a patient with chronic hepatitis c-associated PCT. Therapy with interferon (IFN) transiently decreased HCV rna levels, but levels of urinary porphyrins and serum transaminases and ferritin remained unchanged. serum ferritin and urinary porphyrin levels improved after phlebotomy, but this therapy was not effective in improving serum transaminase levels. Although a physiopathological association between HCV infection and PCT has been suggested previously, IFN was not effective in this patient. The transient decrease in HCV rna levels was a factor independent of porphyrin metabolism.
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ranking = 6
keywords = hepatitis
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5/23. Treatment of porphyria cutanea tarda with phlebotomy in a patient on peritoneal dialysis.

    porphyria cutanea tarda (PCT) is frequently described among patients with chronic renal failure undergoing hemodialysis. One patient who was receiving peritoneal dialysis and had PCT develop has been described in the literature. However, it was later determined that the patient's PCT was related to the hepatotoxic drug she was receiving rather than her peritoneal dialysis. Our patient is the first reported case, to our knowledge, of a patient with end-stage renal disease with negative hepatitis serology who was not receiving a hepatotoxic drug, or on hemodialysis, who had PCT develop while receiving peritoneal dialysis.
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keywords = hepatitis
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6/23. porphyria cutanea tarda and hiv: two cases associated with hepatitis c.

    hepatitis c virus (HCV) is a probable etiologic factor for the development of porphyria cutanea tarda (PCT), a photosensitive skin disease causing blistering, skin fragility, milia, and scarring. In a review of the literature, the hepatitis c status of patients coinfected with hiv and PCT was not known. Two patients with PCT who were seropositive for hiv and HCV are discussed herein. The appropriateness of performing porphyrin studies in patients diagnosed with hiv and photosensitivity and of prompting physicians to test for hiv and HCV infection in individuals who are diagnosed with PCT is discussed. Because hiv has been isolated from cutaneous blister fluid in patients with PCT and hiv, caregivers should be aware of the infection risk associated with the vesicles and erosions in these patients.
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ranking = 5
keywords = hepatitis
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7/23. Development of porphyria cutanea tarda in a hemodialysis patient after reactivation of hepatitis c virus infection.

    Cases of porphyria cutanea tarda (PCT) are occasionally reported in hemodialysis patients. Recently, hepatitis c virus has been recognized as a precipitating factor of PCT. The activity of the liver disease may be critical for the appearance of PCT. In this regard, liver disease reactivation after treatment with interferon alpha (IFN-alpha) is a well-known phenomenon. We report the case of a hemodialysis patient who developed PCT coincidentally with reactivation of liver disease, immediately after treatment with IFN-alpha. Therefore, in the present case, reactivation of hepatitis after IFN-alpha withdrawal could be the triggering factor. The occurrence of a bout of PCT should be considered as a possible complication at the end of IFN-alpha therapy, if reactivation of the disease exists.
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ranking = 6
keywords = hepatitis
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8/23. porphyria cutanea tarda presenting as cicatricial conjunctivitis.

    PURPOSE: To report a case of porphyria cutanea tarda presenting as cicatricial conjunctivitis.DESIGN: observational study.methods: A 31-year-old man presented with bilateral inferior symblepharon, superior tarsal conjunctival scarring and concretions, and recurrent conjunctival and episcleral injection.RESULTS: Four years after initial presentation, the patient developed hepatitis c, and 2 years later blisters on his scalp and hands. Direct immunofluorescence studies of biopsies taken from the palpebral conjunctiva of the right lower lid were negative for cicatricial pemphigoid. A twenty-four hour urine specimen analysis revealed elevated levels of uroporphyrins and polycarboxylated porphyrins, confirming the diagnosis of porphyria cutanea tarda. The patient was treated with repeated phlebotomies and oral hydroxychloroquine, which resulted in a significant decrease in the skin lesions, conjunctival injection, and concretions under the upper lids.CONCLUSIONS: Cicatricial conjunctivitis may be a manifestation of porphyria cutanea tarda.
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ranking = 1
keywords = hepatitis
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9/23. porphyria cutanea tarda, hepatitis c, alcoholism, and hemochromatosis: a case report and review of the literature.

    porphyria cutanea tarda (PCT) is associated with estrogen, certain medications, alcohol abuse, hepatitis viruses, and iron overload. Numerous studies have demonstrated an increased incidence of hepatitis c in patients with PCT; therefore, hepatitis screening should be routinely performed on these patients. On the other hand, although studies have long suspected hereditary hemochromatosis (HH) to be an underlying condition of PCT, many physicians have a low index of suspicion. Also, diagnosis of HH has been difficult until recently, when the gene mutation was identified. We present a case of a patient with PCT, hepatitis c, and alcoholism who was homozygous for the HH gene mutation.
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ranking = 8
keywords = hepatitis
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10/23. porphyria cutanea tarda in pregnancy: a case report.

    porphyria cutanea tarda (PCT) is the most common disorder of porphyria metabolism in europe and north america.The incidence in women appears to be increasing, perhaps as a result of widespread use of hormonal contraception. Treatment in pregnancy is largely supportive, but screening of the pregnant patient for antinuclear antibodies, diabetes, human immunodeficiency virus (hiv) and hepatitis should be performed, as there is an association of PCT with these conditions. The baby should also be screened postpartum for PCT. patients with PCT cope well with normal and operative delivery; however, an anaesthetic plan of management for labour and delivery is advisable.
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ranking = 1
keywords = hepatitis
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