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    2129 results match your criteria Porphyria Cutanea Tarda

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    Hepatitis C Treatment in Patients With Porphyria Cutanea Tarda.
    Am J Med Sci 2017 Jun 8;353(6):523-528. Epub 2017 Mar 8.
    Department of Preventive Medicine and Community Health, University of Texas Medical Branch, Galveston, Texas; Department of Internal Medicine, University of Texas Medical Branch, Galveston, Texas.
    Background: Hepatitis C virus (HCV) infection is a common susceptibility factor for porphyria cutanea tarda (PCT). Experience on HCV treatment in patients with PCT is limited. Recently, HCV treatment has improved with direct-acting antivirals (DAA). Read More

    The effect of sustained virological response on the risk of extrahepatic manifestations of hepatitis C virus infection.
    Gut 2017 Jun 20. Epub 2017 Jun 20.
    Department of Epidemiology, Human Genetics, and Environmental Sciences, The University of Texas School of Public Health, Houston, Texas, USA.
    Background And Aim: Chronic HCV infection is associated with several extrahepatic manifestations (EHMs). Data on the effect of sustained virological response (SVR) on the risk of EHMs are limited.

    Methods: We conducted a retrospective cohort study using data of patients from the US Veterans Affairs HCV Clinical Case Registry who had a positive HCV RNA test (10/1999-08/2009). Read More

    Sporadic Porphyria in a patient with stage II melanoma treated with interferon α.
    Curr Drug Saf 2017 May 2. Epub 2017 May 2.
    CH ST ELOI - DERMATOLOGY MONTPELLIER. France.
    Porphyria cutanea tarda (PCT) is the most common form of human porphyria, due to reduced activity of uroporphyrinogen decarboxylase (UROD). There are many factors which can trigger PCT such as viral infections, excessive alcohol intake, iron overload, hepatotoxic drugs and hepatic tumours. Drug induced PCT is well documented but PCT induced by interferon α has rarely been described and only in cases of Hepatitis C Virus (HCV) infection or haematological malignancies. Read More

    Direct-acting antivirals for hepatitis C virus induce a rapid clinical and biochemical remission of porphyria cutanea tarda.
    Br J Dermatol 2017 Mar 29. Epub 2017 Mar 29.
    Dermatology Unit, Hospital Clinic de Barcelona, Barcelona, Spain.
    Sporadic porphyria cutanea tarda (PCT) is strongly associated with HCV infection in our population (1,2,3,4.) . Therapeutic options for PCT include phlebotomies and low-dose 4-aminoquinolines, which show high rates of disease remission. Read More

    Porphyria cutanea tarda: an intriguing genetic disease and marker.
    Int J Dermatol 2017 Jun 21;56(6):e106-e117. Epub 2017 Mar 21.
    Dermatology, Rutgers New Jersey Medical School, Newark, NJ, USA.
    Porphyrias are a group of intriguing genetic diseases of the heme pathway, of which porphyria cutanea tarda (PCT) is the most common. Resulting from a defect in enzymes in the porphyria pathway, PCT has been linked to several conditions. Recent studies have demonstrated a change in thinking regarding the human immunodeficiency virus (HIV) and development of PCT. Read More

    Performing therapeutic venesection in a doctor's surgery.
    Aust Fam Physician 2017 Mar;46(3):132-138
    Background: 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.

    Objective: This article briefly reviews the pathophysiology of these conditions, and the rationale and goals of therapeutic venesection as a treatment modality. It also summarises the venesection procedure itself and the considerations for setting up a venesection service in a doctor's surgery. Read More

    Hepatitis C virus and its cutaneous manifestations: treatment in the direct-acting antiviral era.
    J Eur Acad Dermatol Venereol 2017 Mar 2. Epub 2017 Mar 2.
    Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NY, USA.
    New all-oral direct-acting antivirals (DAA) have changed the hepatitis C virus (HCV) treatment landscape. Given that dermatologists frequently encounter HCV-infected patients, knowledge of the current treatment options and their utility in treating HCV-associated dermatologic disorders is important. In addition to highlighting the new treatment options, we review four classically HCV-associated dermatologic disorders - mixed cryoglobulinaemia (MC), lichen planus (LP), porphyria cutanea tarda (PCT) and necrolytic acral erythema (NAE) - and examine the role for all-oral direct-acting antiviral (DAA) regimens in their treatment. Read More

    Porphyria Cutanea Tarda in a Patient with End-Stage Renal Disease: A Case of Successful Treatment with Deferoxamine and Ferric Carboxymaltose.
    Case Rep Nephrol 2017 22;2017:4591871. Epub 2017 Jan 22.
    Department of Dermatology, Centro Hospitalar Lisboa Norte, Lisbon, Portugal.
    Porphyria cutanea tarda (PCT) is a rare disease, with a strong association with hepatitis C virus. PCT is particularly problematic in end-stage renal disease patients as they have no renal excretion of porphyrins and these are poorly dialyzed. Also, conventional treatment of PCT is compromised in these patients as hydroxychloroquine is contraindicated, phlebotomies with the stipulated frequency are poorly tolerated in already anaemia-prone patients, and iron-chelating agents are less efficient in removing iron and contribute to worsening anaemia. Read More

    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.
    Abdom Radiol (NY) 2017 Jul;42(7):1813-1818
    Department of Radiology, Faculty of Medicine, Fukuoka University, 7-45-1 Nanakuma, Jonan-ku, Fukuoka, 814-0180, Japan.
    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. Read More

    Bullous, pseudobullous, & pustular dermatoses.
    Semin Diagn Pathol 2017 May 14;34(3):250-260. Epub 2016 Dec 14.
    Section of Dermatopathology, Division of Surgical Pathology & Cytopathology, University of Virginia Medical Center, Charlottesville, VA, United States. Electronic address:
    Several dermatoses are typified by the formation of spaces (blisters; bullae) within or beneath the epidermis. These may be acellular or filled with particular species of inflammatory cells. Etiological categories include infectious, immune-mediated, genetic, drug-related, and idiopathic lesions. Read More

    Photodermatoses: Kids are not just little people.
    Clin Dermatol 2016 Nov - Dec;34(6):724-735. Epub 2016 Jul 12.
    University of Connecticut School of Medicine, Department of Dermatology, Farmington, CT.
    Photodermatoses are a group of skin disorders caused by abnormal reaction to ultraviolet radiation. Photodermatoses are divided into four groups: (1) immunologically mediated photodermatoses; (2) chemical- and drug-induced photodermatoses; (3) photoaggravated dermatoses; and (4) hereditary photodermatoses. This contribution discusses differences in the approach and diagnosis of pediatric and adult patients with suspected photodermatoses, focusing on immunologically mediated photodermatoses and chemical- and drug-induced photodermatoses. Read More

    Hepatic porphyria: A narrative review.
    Indian J Gastroenterol 2016 Nov 31;35(6):405-418. Epub 2016 Oct 31.
    Department of Internal Medicine, UAB University of Alabama in Birmingham, Birmingham, AL, USA.
    Porphyrias are a group of metabolic disorders, which result from a specific abnormality in one of the eight enzymes of the heme biosynthetic pathway. These have been subdivided based on the predominant site of enzyme defect into hepatic and erythropoietic types and based on clinical presentation into acute neurovisceral and cutaneous blistering porphyrias. This review focuses on hepatic porphyrias, which include acute intermittent porphyria (AIP), variegate porphyria (VP), hereditary coproporphyria (HCP), aminolevulinic acid dehydratase deficiency porphyria (ADP), and porphyria cutanea tarda (PCT). Read More

    Acute intermittent porphyria precipitated by atazanavir/ritonavir.
    Int J STD AIDS 2016 Nov 11;27(13):1234-1235. Epub 2016 Feb 11.
    Division of Infectious Diseases, Department of Medicine, Hofstra North Shore-LIJ School of Medicine, Manhasset, NY, USA.
    Porphyrias are a group of metabolic disorders that are relatively uncommon and underdiagnosed. Although the association between HIV infection and antiretrovirals with porphyria cutanea tarda is well established, there are fewer data linking HIV and the acute hepatic porphyrias. We report the first case of acute intermittent porphyria precipitated by the drugs atazanavir and ritonavir, presenting with unexplained abdominal pain. Read More

    The D519G Polymorphism of Glyceronephosphate O-Acyltransferase Is a Risk Factor for Familial Porphyria Cutanea Tarda.
    PLoS One 2016;11(9):e0163322. Epub 2016 Sep 23.
    Department of Medicine, Hematology Division, University of Utah School of Medicine, Salt Lake City, Utah, United States of America.
    Both familial and sporadic porphyria cutanea tarda (PCT) are iron dependent diseases. Symptoms of PCT resolve when iron stores are depleted by phlebotomy, and a sequence variant of HFE (C282Y, c.843G>A, rs1800562) that enhances iron aborption by reducing hepcidin expression is a risk factor for PCT. Read More

    Clinical, Biochemical and Molecular Characteristics of the Main Types of Porphyria.
    Adv Clin Exp Med 2016 Mar-Apr;25(2):361-8
    Department of Haemostasis and Metabolic Disorders of the Institute of Haematology and Transfusion Medicine, Warszawa, Poland.
    Porphyrias are diverse disorders that arise from various inherited enzyme defects in the heme biosynthesis pathway, except for porphyria cutanea tarda (PCT), in which the enzyme deficiency in most cases is acquired. The biosynthetic blocks resulting from the defective enzymes are largely expressed either in the liver or bone marrow, the sites where the majority of heme is produced. Although the pathophysiologic mechanisms of the clinical manifestations of the porphyrias are not fully understood, two cardinal features prevail: skin photosensitivity and neurologic symptoms of intermittent autonomic neuropathy, acute neurovisceral attacks, and disorders of the nervous system. Read More

    Porphyria cutanea tarda in a HIV- positive patient.
    An Bras Dermatol 2016 Jul-Aug;91(4):520-3
    Pontifícia Universidade Católica do Paraná (PUCPR) - Curitiba (PR), Brazil.
    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. Read More

    Clinical applications of therapeutic phlebotomy.
    J Blood Med 2016 18;7:139-44. Epub 2016 Jul 18.
    Department of Physical Medicine and Rehabilitation, Soonchunhyang University, Cheonan Hospital, Cheonan, South Korea.
    Phlebotomy is the removal of blood from the body, and therapeutic phlebotomy is the preferred treatment for blood disorders in which the removal of red blood cells or serum iron is the most efficient method for managing the symptoms and complications. Therapeutic phlebotomy is currently indicated for the treatment of hemochromatosis, polycythemia vera, porphyria cutanea tarda, sickle cell disease, and nonalcoholic fatty liver disease with hyperferritinemia. This review discusses therapeutic phlebotomy and the related disorders and also offers guidelines for establishing a therapeutic phlebotomy program. Read More

    [Treatment by bloodletting in the past and present].
    Srp Arh Celok Lek 2016 Mar-Apr;144(3-4):240-8
    Introduction: Therapeutic bloodletting has been practiced at least 3000 years as one of the most frequent methods of treatment in general, whose value was not questioned until the 19th century, when it was gradually abandoned in Western medicine, while it is still practiced in Arabic and traditional Chinese medicine.

    Content: In modern medicine bloodletting is practiced for very few indications. Its concept was modeled on the process of menstrual bleeding, for which it was believed to"purge women of bad humours. Read More

    Novel Treatment Using Cimetidine for Erythropoietic Protoporphyria in Children.
    JAMA Dermatol 2016 Nov;152(11):1258-1261
    Department of Dermatology, Stanford University, Stanford, California.
    Importance: Erythropoietic protoporphyria (EPP) is a rare hereditary disease of heme biosynthesis that manifests as severe photosensitivity and hepatotoxicity. There have been no effective treatments to date. Cimetidine has been shown to inhibit heme biosynthesis and results in symptomatic improvement in patients with acute intermittent porphyria (AIP) and porphyria cutanea tarda (PCT). Read More

    Human Immunodeficiency Virus Associated Sporadic Nonfamilial Porphyria Cutanea Tarda.
    Indian J Dermatol 2016 May-Jun;61(3):318-20
    Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal, India.
    Porphyria cutanea tarda (PCT), a relatively uncommon metabolic disease, is the most common cutaneous porphyria. Here, we present the case of a patient diagnosed with sporadic, nonfamilial PCT that presented with classical cutaneous findings and multiple risk factors, including alcohol abuse, human immunodeficiency virus/AIDS, that have been strongly associated with the sporadic form of PCT. Read More

    Reflectance confocal microscopy in the diagnosis of vesicobullous disorders: case series with pathologic and cytologic correlation and literature review.
    Skin Res Technol 2016 Nov 4;22(4):479-486. Epub 2016 Jun 4.
    Dermatology Clinic, University of Catania, Catania, Italy.
    Background: Vesicobullous disorders are characterized by intraepidermal or subepidermal blistering resulting from different pathogenetic mechanisms. The diagnosis is generally based on clinical examination and semi-invasive/invasive procedures such as cytology and histopathology. In vivo reflectance confocal microscopy (RCM) is a non-invasive technique for real-time, en face imaging of the epidermis and upper dermis with high resolution close to conventional histopathology. Read More

    Cutaneous Porphyrias: Causes, Symptoms, Treatments and the Danish Incidence 1989-2013.
    Acta Derm Venereol 2016 Nov;96(7):868-872
    Department of Clinical Biochemistry and Pharmacology, Odense University Hospital, 5000 Odense C, Denmark.
    Porphyrias are rare diseases caused by altered haem synthesis leading to the accumulation of different haem intermediates. Neurovisceral attacks may occur in acute porphyrias, while photosensitivity is the presenting symptom in cutaneous porphyrias. We present here an overview of symptoms and a flowchart for the diagnosis of cutaneous porphyrias, with recommendations for monitoring and an update of treatment options. Read More

    Extrahepatic Manifestations of Hepatitis C: A Meta-analysis of Prevalence, Quality of Life, and Economic Burden.
    Gastroenterology 2016 Jun 26;150(7):1599-608. Epub 2016 Feb 26.
    Center for Outcomes Research in Liver Diseases, Washington, District of Columbia.
    Background & Aims: Hepatitis C virus (HCV) infection has hepatic and extrahepatic manifestations with various costs and impairments to health-related quality of life (HRQL). We performed a meta-analysis to determine the prevalence of extrahepatic manifestations in patients with HCV infection, how these impair HRQL, and their costs.

    Methods: We performed systematic reviews of the literature using MEDLINE, CINAHL, and the Cochrane Systematic Review Database, from 1996 through December 2014, to identify studies of the following extrahepatic manifestations of HCV infection: mixed cryoglobulinemia, chronic kidney or end-stage renal disease, type 2 diabetes, B-cell lymphoma, lichen planus, Sjögren's syndrome, porphyria cutanea tarda, rheumatoid-like arthritis, or depression. Read More

    Porphyria cutanea tarda associated with HFE C282Y homozygosity, iron overload, and use of a contraceptive vaginal ring.
    J Community Hosp Intern Med Perspect 2016 17;6(1):30380. Epub 2016 Feb 17.
    Department of Medicine, Intermountain Medical Center, University of Utah, Salt Lake City, UT, USA.
    Porphyria cutanea tarda (PCT) is characterized by decreased uroporphyrinogen decarboxylase activity in hepatocytes, uroporphyrin I and heptacarboxyl porphyrin III accumulation, photosensitivity dermatitis, and increased storage iron. In women, estrogen therapy, including oral contraceptives, postmenopausal hormone replacement, and tamoxifen for breast cancer treatment, is a risk factor for PCT. We report the case of a woman who presented with PCT, HFE C282Y homozygosity, and hepatic iron overload and was using a contraceptive vaginal ring containing ethinyl estradiol, an estrogen. Read More

    [Porphyria cutanea tarda: the benefit of additional diagnostics].
    Ned Tijdschr Geneeskd 2016 ;160:A9166
    IJsselland Ziekenhuis, Capelle aan den IJssel.
    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. Read More

    Illness Perception and Psychological Distress in Persons with Porphyria Cutanea Tarda.
    Acta Derm Venereol 2016 Jun;96(5):674-8
    Department of Global Health and Primary Health Care, University of Bergen P.b. 7804, 5020 Bergen, Norway.
    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. Read More

    [Porphyria cutanea tara].
    Hautarzt 2016 Mar;67(3):207-10
    Hautklinik - Klinik für Dermatologie & Allergologie, RWTH Aachen University, Aachen, Deutschland.
    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. Read More

    Cutaneous manifestations of hepatitis C in the era of new antiviral agents.
    World J Hepatol 2015 Nov;7(27):2740-8
    Simone Garcovich, Rodolfo Capizzi, Department of Dermatology, Policlinico A. Gemelli, Catholic University of Sacred Heart, 00168 Rome, Italy.
    The association of chronic hepatitis C virus (HCV) infection with a wide spectrum of cutaneous manifestations has been widely reported in the literature, with varying strength of epidemiological association. Skin diseases which are certainly related with chronic HCV infection due to a strong epidemiological and pathogenetic association are mixed cryoglobulinemia, lichen planus and porphyria cutanea tarda. Chronic pruritus and necrolytic acral erythema are conditions that may share a possible association with HCV infection, while several immune-mediated inflammatory skin conditions, such as psoriasis, chronic urticaria and vitiligo, have been only anecdotally reported in the setting of chronic HCV infection. Read More

    Drug induced pseudoporphyria in CKD: A case report.
    Indian J Nephrol 2015 Sep-Oct;25(5):307-9
    Department of Medicine, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India.
    Pseudoporphyria (PP) is used to describe a photodistributed bullous disorder with clinical and histologic features of porphyria cutanea tarda (PCT) but without accompanying biochemical porphyrin abnormalities. Medications, excessive sun and ultraviolet radiation exposure, have all been reported to develop PP. We report a case of PP in a 49-year-old man with CKD stage 3a, caused due to torsemide intake. Read More

    Human immunodeficiency virus and risk of porphyria cutanea tarda: a possible association examined in a large hospital.
    Photodermatol Photoimmunol Photomed 2016 Mar 15;32(2):93-7. Epub 2015 Dec 15.
    Biochemistry and Molecular Genetics Department, Hospital Clínic, IDIBAPS, University of Barcelona, Barcelona, Spain.
    Background: Human immunodeficiency virus (HIV) infection has been reported to be a risk factor for porphyria cutanea tarda (PCT).

    Purpose: To assess the strength of the association between HIV infection and PCT in a large hospital setting.

    Methods: All patients (N = 210) diagnosed of PCT between 1990 and 2014 were retrospectively investigated for HIV infection and co-precipitating factors. Read More

    Phototoxicity, Pseudoporphyria, and Photo-onycholysis Due to Voriconazole in a Pediatric Patient With Leukemia and Invasive Aspergillosis.
    J Pediatric Infect Dis Soc 2015 Jun 4;4(2):e22-4. Epub 2014 Jul 4.
    Infectious Diseases Division, Department of Pediatrics.
    Voriconazole is a triazole antifungal agent superior to amphotericin B in the treatment of invasive aspergillosis. It is generally well tolerated and has excellent oral bioavailability, providing significant benefit in the treatment of invasive fungal infections. There have been numerous reports of dermatologic reactions to this agent, including erythroderma, cheilitis, Stevens-Johnson syndrome, discoid lupus erythematosus, pseudoporphyria, squamous cell carcinoma, and photosensitivity reactions. Read More

    Dermatologic Extrahepatic Manifestations of Hepatitis C.
    J Clin Transl Hepatol 2015 Jun 15;3(2):127-33. Epub 2015 Jun 15.
    Department of Medicine, Division of Gastroenterology-Hepatology, University of Connecticut Heath Center, Farmington, CT, USA.
    Hepatitis C virus (HCV) affects millions of people worldwide, and an estimated 3.2 million people in the United States. HCV is a hepatotropic and lymphotropic virus that causes not only liver disease, but also a significant number of extrahepatic manifestations (EHMs). Read More

    Hem12, an enzyme of heme biosynthesis pathway, is monoubiquitinated by Rsp5 ubiquitin ligase in yeast cells.
    Acta Biochim Pol 2015 28;62(3):509-15. Epub 2015 Aug 28.
    Institute of Biochemistry and Biophysics Polish Academy of Sciences, Warsaw, Poland.
    Heme biosynthesis pathway is conserved in yeast and humans and hem12 yeast mutants mimic porphyria cutanea tarda (PCT), a hereditary human disease caused by mutations in the UROD gene. Even though mutations in other genes also affect UROD activity and predispose to sporadic PCT, the regulation of UROD is unknown. Here, we used yeast as a model to study regulation of Hem12 by ubiquitination and involvement of Rsp5 ubiquitin ligase in this process. Read More

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