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Porphyria Cutanea Tarda - Top 30 Publications

Porphyria.

Performing therapeutic venesection in a doctor's surgery.

Although venesection was widely applied in the past for the treatment of various ailments and diseases, in modern medical practice, it is indicated in very few conditions, namely, hereditary haemochromatosis, polycythaemia and porphyria cutanea tarda.

A case of porphyria cutanea tarda of the liver exhibiting multifocal macrovesicular steatosis in the background of microvesicular steatosis, probably caused by uneven iron accumulation.

A 61-year-old man with chronic hepatitis B and a history of alcohol overconsumption was admitted to our hospital for the scrutiny of multiple echogenic liver nodules. CT and hepatobiliary phase of gadoxetate-enhanced MR imaging revealed no nodular lesions. Quantitative fat fraction images and R2* map of MR imaging suggested homogeneous steatosis and uneven iron deposition in the liver, namely moderately and severely elevated R2* values at the nodules and surrounding background liver, respectively. Biopsy specimens showed macrovesicular fatty liver and less iron deposition at the echogenic nodules, and microvesicular fatty change and more prominent iron deposition at the surrounding liver tissue. The patient's urinary uroporphyrin level was elevated, and the final diagnosis of porphyria cutanea tarda was made. In patients with history of excessive alcohol intake or viral hepatitis, echogenic nodules on ultrasonography along with radiological evidence of absence of space occupying lesions, and presence of excessive intrahepatic fat and iron, might suggest a possible diagnosis of porphyria cutanea tarda.

Painless Vesicular Eruption on the Dorsal Surfaces of the Hands.

Porphyria Cutanea Tarda.

Porphyria cutanea tarda in a HIV- positive patient.

This is a case report about Porphyria cutanea tarda (PCT) and its relationship with the infection caused by the human immunodeficiency virus (HIV). Cutaneous porphyria is an illness caused by enzymatic modification that results in partial deficiency of uroporphyrinogen decarboxylase (Urod), which may be hereditary or acquired. Several studies suggest that HIV infection associated with cofactors might trigger the development of porphyria cutanea tarda. In this case report, we present a patient infected with HIV, who after the introduction of antiretroviral therapy (ART) enjoyed clinical improvement of porphyria cutanea tarda symptoms.

Porphyria cutanea tarda: the benefit of additional diagnostics.

The porphyrias are a clinically and genetically heterogeneous group of relatively rare metabolic diseases that result from disorders in the biosynthesis of haeme. Porphyria cutanea tarda (PCT) is the most common type, accounting for 80-90% of all porphyrias, and is essentially an acquired disease, although PCT can also occur on a familial basis. We describe a 71-year-old female and a 62-year-old male patient, both of whom had several risk factors for developing PCT, ranging from iron overload due to a mutation in the hereditary haemochromatosis protein (HFE) gene, alcohol use, smoking, and exogenous oestrogen, to persistent hepatitis C infection. The clinical relevance of the several diagnostic modalities is important in PCT. Diagnostic evaluation is important in order to confirm the diagnosis, but also to evaluate the treatment response in the context of long-term follow-up in the prevention of late complications of PCT, i.e. hepatocellular carcinoma.

Illness Perception and Psychological Distress in Persons with Porphyria Cutanea Tarda.

Porphyria cutanea tarda (PCT) requires long-term treatment and follow-up, although many patients experience life-long remission. The aim of this cross-sectional postal survey was to describe and investigate the association between illness perception, health complaints, self-reported symptoms and distress in persons with PCT. The participants perceived PCT as a chronic condition with high levels of personal and treatment control. Persons who reported active symptoms scored higher on perceived illness threat, total health complaints and psychological distress compared with those in remission or latent phases. However, a higher perception of illness threat and the total burden of health complaints were more closely associated with psychological distress than were perceived PCT symptoms activity. This has implications for clinical consultation; dermatologists should be attentive to symptoms activity, but also recognize that patients in remission with a high perceived illness threat and multiple health complaints might be especially vulnerable to psychological distress with regards to PCT.

Porphyria cutanea tara.

Porphyria cutanea tara (PCT) has a prevelance of about 40 new diagnoses per 1 million people per year and is the most frequently occurring type of porphyria worldwide. Inhibition of the uroporphyrinogen decarboxylase (UROD) is the main cause of the disease, which can be the result of a heterozygous or homozygous mutation of the UROD gene; however, xenobiotics or other diseases may play an important role for the precipitation of the disease. Risk factors include alcohol, estrogen, iron overload, and hemochromatosis, hepatitis C or poisoning, e.g., with polyhalogenated aromatic compounds such as hexachlorobenzene. Signs and symptoms are blisters, skin fragility, erosions hyperpigmentation, sclerodermoid plaques. Therapy includes sun protection, prevention of risk factors, phlebotomy, and chloroquine.

HIV infection and porphyria cutanea tarda, report of a case.

Sclerodermiform porphyria cutanea tarda after torasemide.

Human immunodeficiency virus and risk of porphyria cutanea tarda: a possible association examined in a large hospital.

Human immunodeficiency virus (HIV) infection has been reported to be a risk factor for porphyria cutanea tarda (PCT).

Victorian Vampires Validated--The Similarities Between a Legendary Creature and a Dermatologic Pathology.

Porphyria cutanea tarda in a child with acute lymphoblastic leukemia.

Sclerodermoid lesions in a patient with multiple transplants and porphyria cutanea tarda.

Patients with chronic graft versus host disease may exhibit a range of sclerotic features. Herein we present a patient with confirmed porphyria cutanea tarda who subsequently developed chronic graft versus host disease.

Medicine-caused itch, wounds and bullous skin in three patients with pseudoporphyria.

Pseudoporphyria cutanea tarda is a well described bullous skin disorder which resembles porphyria cutanea tarda. However, the levels of porphyrins in plasma, urine and faeces are normal. We present three cases of patients with classical symptoms of pseudoporphyria. Two of the patients developed pseudoporphyria after the combination of intensive sunbathing and medications well known to cause pseudoporphyria. The third case received haemodialysis and furosemide.

X-linked dominant protoporphyria: response to "Cutaneous porphyrias part 1".

Allergic to the sun.

Treatment of chronic hepatitis with boceprevir leads to remission of porphyria cutanea tarda.

Pseudoporphyria following self-medication with chlorophyll.

Two cases of pseudoporphyria are described in which the clinical features of porphyria cutanea tarda occurred in the absence of abnormalities in porphyrin metabolism. Both patients presented with skin fragility and bullae on the dorsal aspect of the hands. The patients consumed a commercial liquid chlorophyll drink in which we detected fluorescent compounds with characteristics typical of previously described chlorophyll derived photosensitisers.

Porphyria cutanea tarda and Sjogren's syndrome.

Porphyria cutanea tarda is prevalent in connective tissue disease, common in systemic lupus erythematosus. However, the co-existence of primary sjogren's syndrome and porphyria cutanea tarda is rare and poses diagnostic and therapeutic challenges. We report a case of porphyria cutanea tarda associated with primary sjogren's syndrome.

A woman with hypertrichosis.

A skin disease, a blood disease or something in between? An exploratory focus group study of patients' experiences with porphyria cutanea tarda.

Porphyria cutanea tarda (PCT) is characterized by fragile skin with blistering on sun-exposed areas. Symptoms typically develop in late adulthood and can be triggered by iron overload, alcohol intake, oestrogens and various liver diseases. Treatment consists of phlebotomy to reduce iron, or increasing urinary porphyrin excretion by administering chlorochin. To optimize patient care, health personnel need to understand the subjective experiences of PCT.

Ocular manifestations in porphyria cutanea tarda.

A 24-year-old man presented with pain, sticky discharge and loss of vision in the right eye. He has had typical skin manifestations of porphyria cutanea tarda (PCT) since 6 years and ophthalmological symptom for 6 weeks. On ophthalmological examination, visual acuity was light perception in the right eye and 6/12 in the left. There were bilateral, symmetrical temporal scleromalacia along with temporal corneal melting in both eyes and perforation in the right eye. Ultrasonography B-scan (USG B-scan) revealed a retinal detachment in the right eye. Artificial tear instillation was started every hour along with topical antibiotic coverage in both eyes. Additionally, ultraviolet protective sunglasses and hat for photo-protection was advised. The vision in the right eye improved to 5/60 along with subsidence of retinal detachment on repeat USG B-scan after 3 weeks.

Woman with facial hypertrichosis.

Porphyrias in Norway.

Porphyria is an umbrella term for a group of largely hereditary diseases that are due to defective haem synthesis. The diseases have a varied and partly overlapping range of symptoms and presentations. The commonest forms of porphyria are porphyria cutanea tarda, acute intermittent porphyria and erythropoietic protoporphyria. The purpose of this study is to provide an overview of the prevalence and pathological manifestations of porphyrias in Norway.

Porphyria cutanea tarda in a child following multi-agent chemotherapy.

Porphyria cutanea tarda (PCT) is a blistering skin disorder that occurs most commonly in middle-aged individuals. It is caused by decreased uroporphyrinogen decarboxylase (UROD) activity, which results in elevated levels of uroporphyrinogen. Occurrence remains very rare in children with some sources quoting as few as 50 reports of childhood cases.1 The literature reports occasional cases of PCT onset with various drugs, including barbiturates, estrogens, griseofulvin, rifampicin, sulfonamides, imatinib, methotrexate, tamoxifen, and cyclophosphamide, however its incidence in childhood is uncommon.2-6 We present a case of new-onset PCT in an eight year-old following treatment of pre-B cell acute lymphoblastic leukemia with multi-agent chemotherapy.

Porphyria cutanea tarda with sclerodermatous changes and hemochromatosis.

A 55-year-old woman presented with blistering on the back of her hands and shiny, thickened skin in her décolletage. Laboratory examination revealed increased urinary total and high carboxylated porphyrins and homozygosity for mutation C282Y in the HFE gene. Histopathology showed thickened collagen fibers in the presternal region. Based on these findings we made the diagnosis of porphyria cutanea tarda with pseudoscleroderma and hemochromatosis. Pseudoscleroderma is a rare complication of PCT but can also constitute the first cutaneous symptom of the disease, leading the way to diagnosis. Usually, adequate treatment of PCT with normalization of porphyrin values also results in improvement of pseudoscleroderma.

The case | A hemodialysis patient with bullous skin lesions.

Sporadic porphyria cutanea tarda: treatment with chloroquine decreases hyperglycemia and reduces development of metabolic syndrome.