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The text is the summary of recent articles on porphyria cutanea tarda at 75 thresold from National Library of Medicine (NLM). This information is subject to NCBI's Disclaimer and Copyright notice.
Acute intermittent porphyria causes Kidney injury, whereas Medical situations associated with end-stage Renal disease, such as porphyrin accumulation, iron overload and hepatitis c, participate in the inhibition of uroporphyrinogen decarboxylase and predispose the Individual to porphyria cutanea tarda . There are no studies concerning the genetic ancestry of patients with porphyria cutanea tarda from a mixed population . Thirty patients living in Rio de Janeiro with sporadic porphyria cutanea tarda were studied for the genetic ancestry through informative markers - INDELS . Biopsy of her Bullous skin lesions was consistent with porphyria cutanea tarda, as were her serological and urinary exams . This case identifies porphyria cutanea tarda as an important differential diagnosis for the rheumatologist to consider when evaluating patients with Bullous skin lesions .
Different tests are available for diagnosing and screening for type II diabetes mellitus, however choosing the most suitable test is challenging . The pitfalls in the different tests along with the interfering comorbidities and treatments concerning patients with porphyria cutanea tarda complicate diagnosing these patients with diabetes mellitus . porphyria cutanea tarda, bullous pemphigoid, epidermolysis bullosa, and pseudoporphyria are other differential diagnoses of Bullous lesions, and they must be excluded . CASE REPORT We present a 42-year-old African American male with long standing poorly controlled insulin dependent diabetes mellitus with blisters on his left Hand and Feet . Some of the porphyrias that cause blistering (usually bullae) and fragility (clinically and histologically identical to porphyria cutanea tarda) can also be associated with acute neurovisceral porphyria attacks, particularly variegate porphyria and hereditary coproporphyria .
Management of porphyria cutanea tarda mainly consists of visible-light photoprotection measures while awaiting the effects of treating the underlying liver disease (if possible) and treatments to reduce serum iron and porphyrin levels . This article reviews the clinical presentation with emphasis on cutaneous disease, etiopathogenesis, diagnosis, and treatment options available for the different forms of scleroderma firstly and for scleroderma-like disorders, including scleromyxedema, scleredema, nephrogenic Systemic fibrosis, eosinophilic fasciitis, Chronic graft-versus-host disease, porphyria cutanea tarda, diabetic stiff-hand syndrome (diabetic cheiroartropathy), and other minor forms . This latter group of conditions, termed also scleroderma mimics, sclerodermiform diseases, or pseudosclerodermas, shares the common thread of skin thickening but presents with distinct cutaneous manifestations, skin histology, and Systemic implications or disease associations, differentiating each entity from the others and from scleroderma . Porphyria cutanea tarda is the most common form of porphyria, and it is thought that HCV interferes with iron stores, which can promote porphyria cutanea tarda . Primary or autoimmune photodermatoses: Polymorphic light eruption .
Exogenous or drug/chemical-induced photodermatoses: Drug-induced photosensitivity: common photosensitizing drugs are thiazides, tetracyclines, non-steroidal anti-inflammatory drugs (NSAIDs), phenothiazines, voriconazole, quinine, vemurafenib, and many others . Genetic photodermatoses (very rare disorders due to genomic instability): xeroderma pigmentosum . Before commencing therapeutic venesection, management goals in terms of laboratory parameters should be set for Individual patients . 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 . PCT is particularly problematic in end-stage Renal disease patients as they have no Renal excretion of porphyrins and these are poorly dialyzed .
This case suggests that deferoxamine is efficient in treatment of porphyria cutanea tarda . Examples of such disorders include impetigo, Herpes virus infections, pemphigus, bullous pemphigoid and pemphigoid gestationis, epidermolysis bullosa acquisita, IgA-related dermatoses, inherited epidermolysis bullosa variants, Hailey-Hailey disease, and porphyria cutanea tarda . Other conditions manifest microscopic acantholysis within the surface epithelium but are not associated with clinical bullae, such as darier disease and Grover disease . Phlebotomy and chloroquine, which are usually effective in treating familial porphyria cutanea tarda, are generally less effective in individuals with HEP . Prevention of primary manifestations: Protection from sunlight .
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) . 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 . A link with the HCV infection is considered to be established with membranoproliferative glomerulonephritis, leukocytoclastic vasculitis, lymphoproliferative disorders (in particular B cell lymphoma), Sjögren and sicca syndrome, lichen planus, porfyria cutanea tarda and diabetes mellitus .
References: 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 ,Press Refresh to fetch fresh content with references from pubmed. This content was cached on 2018-04-16 comments powered by Disqus
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