Porphyria Cutanea Tarda Symptomatica
in [Lupus]
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From: ironjustice on 23 Aug 2008 16:29 This is age-related iron accumulation .. **acquired** .. porphyria cutanea tarda .. No .. "genetics" .. involved .. Just plain old .. iron **accumulation** .. also known as .. Porphyria Cutanea Tarda **Symptomatica** http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1928842 Can Med Assoc J. 1965 September 4; 93(10): 537540. PMCID: PMC1928842 Acquired Porphyria Cutanea Tarda Report of a Case Successfully Treated by Phlebotomy Andrew Koval, C. W. E. Danby, and H. Petermann . Abstrac tCurrently, the porphyrias are classified in four main groups: congenital porphyria, acute intermittent porphyria, porphyria cutanea tarda hereditaria, and porphyria cutanea tarda symptomatica. The acquired form of porphyria (porphyria cutanea tarda symptomatica) occurs in older males and is nearly always associated with chronic alcoholism and hepatic cirrhosis. The main clinical changes are dermatological, with excessive skin fragility and photosensitivity resulting in erosions and bullae. Biochemically, high levels of uroporphyrin are found in the urine and stools. Treatment to date has been symptomatic and usually unsuccessful. A case of porphyria cutanea tarda symptomatica is presented showing dramatic improvement of both the skin lesions and porphyrin levels in urine and blood following repeated phlebotomy. Possible mechanisms of action of phlebotomy on porphyria cutanea tarda symptomatica are discussed. Who loves ya. Tom Jesus Was A Vegetarian! http://tinyurl.com/634q5a Man Is A Herbivore! http://tinyurl.com/4rq595 DEAD PEOPLE WALKING http://tinyurl.com/zk9fk
From: ironjustice on 24 Aug 2008 00:19 On Aug 23, 1:29 pm, "ironjust...(a)aol.com" <ironjust...(a)aol.com> wrote:excessive skin fragility and photosensitivity resulting in erosions and bullae << The association between iron overload and iron deficiency seems to be .. paradoxal. Hemolytic anemia is diagnosed just before she .. is .. dead .. it seems. Rapidly progressing purpuric lesions to massive hemorrhagic bullae, with rapid improvement by Prednisolon as a coetaneous manifestation of Systemic Lupus Erythematosus: a case report. Khorvash FF, Emami Naeini AA, Behjati MM, Karimifar MM, Khorvash FF, Dialami KK Cases J 2008 Aug 8; 1(1):79. ABSTRACT: BACKGROUND: Systemic lupus Erythematosus is a chronic autodestructive disease, with loss of immune tolerance to nucleic acid and other cross reactive antigens. Despite of the numerous studies, the presence of some new manifestations indicates the greater proportion of unknown data. CASE PRESENTATION: Our case, is a 26-year-old female, by the chief complaint of headache, vomiting, fever and arthralgia. Some hemorrhagic ulcers in her mouth with fulminant pethechia/purpura on her limbs and buttocks were prominent. On admission, she was in hypotensive state. By the clinical suspicion to meningococcal septicemia, lumbar puncture was performed, and antibiotic therapy was started. Cerebrospinal fluid was normal. Suddenly, on the 3rd day of admission, hemorrhagic bullae were evolved from those purpuric lesions. Leukocytosis, immune hemolytic anemia, thrombocytopenia and high antinuclear antibody/double stranded DNA level and hypocomplemania were present simultaneously. In skin biopsy, immune complex deposition in dermoepidermal junction was seen. The diagnosis of Systemic lupus Erythematosus was made. The patient responded well to corticosteroid therapy. CONCLUSION: Coetaneous manifestations are very common in Systemic lupus Erythematosus, and help the physician making differential diagnoses and proper diagnosis. The rapidly evolving hemorrhagic bulla from primary purpuric lesions, with rapid response to Prednisolon, is a rare manifestation of Systemic lupus Erythematosus, which should be considered in such a disease setting. Cases journal [Cases J] -------------------------------------------------------------------------------- Porphyria cutanea tarda in pre-existent lupus erythematosus - is there an association? van Tuyll van Serooskerken AM, Habets JM, Badeloe S, Poblete-Gutiérrez P, Frank J Int J Dermatol 2007 Nov.:50-2. In lupus erythematosus (LE), vesicles and bullae are only rarely seen. However, in some instances such efflorescences might suggest an association with distinct cutaneous diseases, including erythema multiforme, toxic epidermal necrolysis or autoimmune blistering disorders such as bullous pemphigoid, pemphigus vulgaris, and dermatitis herpetiformis Duhring. Another blistering disease that has been described in association with cutaneous and systemic LE is porphyria cutanea tarda (PCT). PCT is a metabolic disorder caused by a deficiency of the fifth enzyme in heme biosynthesis, uroporphyrinogen decarboxylase. Here, we report on a 57-year-old Caucasian woman of Dutch origin with a medical history of mild cutaneous LE who developed skin fragility, blistering skin lesions, milia, and facial hypertrichosis. Subsequent porphyrin analysis in urine and feces confirmed the suspected simultaneous manifestation of LE and PCT. Who loves ya. Tom Jesus Was A Vegetarian! http://tinyurl.com/634q5a Man Is A Herbivore! http://tinyurl.com/4rq595 DEAD PEOPLE WALKING http://tinyurl.com/zk9fk
From: jay on 25 Aug 2008 19:28 > This is age-related iron accumulation .. **acquired** .. porphyria > cutanea tarda .. > > No .. "genetics" .. involved .. Non-ahr gene susceptibility Loci for porphyria and liver injury induced by the interaction of 'dioxin' with iron overload in mice. Among the actions of 2,3,7,8-tetrachlorodibenzo-p-dioxin (dioxin) in mice is the induction of hepatic porphyria. This is similar to the most common disease of this type in humans, sporadic porphyria cutanea tarda (PCT). Evidence is consistent with the actions of dioxin being mediated through binding to the aryl hydrocarbon receptor (AHR) with different Ahr alleles in mouse strains apparently accounting for differential downstream gene expression and susceptibility. However, studies of dioxin-induced porphyria and liver injury indicate that the mechanisms must involve interactions with other genes, perhaps associated with iron metabolism. We performed a quantitative trait locus (QTL) analysis of an F(2) cross between susceptible C57BL/6J (Ahr(b1) allele) and the highly resistant DBA/2 (Ahr(d) allele) strains after treatment with dioxin and iron. For porphyria we found QTLs on chromosomes 11 and 14 in addition to the Ahr gene (chromosome 12). Studies with C57BL/6.D2 Ahr(d) mice confirmed that the Ahr(d) allele alone did not completely negate the response. SWR mice are syngenic for the Ahr(d) allele with the DBA/2 strain but are susceptible to porphyria after elevation of hepatic iron. Analysis of SWRxD2 F(2) mice treated with iron and dioxin showed a QTL on chromosome 11, as well as finding other loci on chromosomes 1 (and possibly 9), for both porphyria and liver injury. These findings show for the first time the location of genes, other than Ahr, that modulate the mechanism of hepatic porphyria and injury caused by dioxin in mice. Orthologous loci may contribute to the pathogenesis of human sporadic PCT. PMID: 11854449
From: ironjustice on 25 Aug 2008 22:28 On Aug 25, 4:28 pm, jay <jaym1...(a)hotmail.com> wrote: snip << One might wonder what you are doing on a thread about NON GENETIC porphyria .. ? Seems your .. contribution is rife with .. genes .. ? There are a number of different porphyria cutanea tardas .. and the REST have .. genes involved .. Why don't you stick with .. genetics .. WHEN you post your .. genetic .. susceptibility .. ? Because .. ? You don't know the difference between non genetic or .. genetic .. ? That could be it couldn't it .. jay .. You may be too stupid to realize what you post .. ? You haven't really showed any kind of intelligence .. yet .. Why don't you keep your stupidity .. confined .. to your OWN threads .. Who loves ya. Tom Jesus Was A Vegetarian! http://tinyurl.com/634q5a Man Is A Herbivore! http://tinyurl.com/4rq595 DEAD PEOPLE WALKING http://tinyurl.com/zk9fk > > This is age-related iron accumulation .. **acquired** .. porphyria > > cutanea tarda .. > > > No .. "genetics" .. involved .. > > Non-ahr gene susceptibility Loci for porphyria and liver injury > induced by the interaction of 'dioxin' with iron overload in mice. > Among the actions of 2,3,7,8-tetrachlorodibenzo-p-dioxin (dioxin) in > mice is the induction of hepatic porphyria. This is similar to the > most common disease of this type in humans, sporadic porphyria cutanea > tarda (PCT). Evidence is consistent with the actions of dioxin being > mediated through binding to the aryl hydrocarbon receptor (AHR) with > different Ahr alleles in mouse strains apparently accounting for > differential downstream gene expression and susceptibility. However, > studies of dioxin-induced porphyria and liver injury indicate that the > mechanisms must involve interactions with other genes, perhaps > associated with iron metabolism. We performed a quantitative trait > locus (QTL) analysis of an F(2) cross between susceptible C57BL/6J > (Ahr(b1) allele) and the highly resistant DBA/2 (Ahr(d) allele) > strains after treatment with dioxin and iron. For porphyria we found > QTLs on chromosomes 11 and 14 in addition to the Ahr gene (chromosome > 12). Studies with C57BL/6.D2 Ahr(d) mice confirmed that the Ahr(d) > allele alone did not completely negate the response. SWR mice are > syngenic for the Ahr(d) allele with the DBA/2 strain but are > susceptible to porphyria after elevation of hepatic iron. Analysis of > SWRxD2 F(2) mice treated with iron and dioxin showed a QTL on > chromosome 11, as well as finding other loci on chromosomes 1 (and > possibly 9), for both porphyria and liver injury. These findings show > for the first time the location of genes, other than Ahr, that > modulate the mechanism of hepatic porphyria and injury caused by > dioxin in mice. Orthologous loci may contribute to the pathogenesis of > human sporadic PCT. PMID: 11854449
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