Porphyria Cutanea Tarda

An autosomal dominant or acquired porphyria due to a deficiency of UROPORPHYRINOGEN DECARBOXYLASE in the LIVER. It is characterized by photosensitivity and cutaneous lesions with little or no neurologic symptoms. Type I is the acquired form and is strongly associated with liver diseases and hepatic toxicities caused by alcohol or estrogenic steroids. Type II is the familial form.

Networked: 780 relevant articles (31 outcomes, 37 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Porphyrias
2.	Erythropoietic Protoporphyria
3.	Iron Overload
4.	Infection
5.	Urticaria (Hives)

Experts

1.	Phillips, J D: 7 articles (04/2009 - 11/2000)
2.	Bonkovsky, Herbert L: 6 articles (01/2013 - 04/2002)
3.	Kushner, J P: 6 articles (01/2009 - 11/2000)
4.	Anderson, Karl E: 5 articles (12/2012 - 02/2002)
5.	Stölzel, U: 5 articles (12/2010 - 11/2000)
6.	Herrero, Carmen: 5 articles (11/2010 - 02/2002)
7.	Frank, Jorge: 5 articles (10/2010 - 06/2004)
8.	Gorman, Nadia: 5 articles (12/2007 - 04/2002)
9.	Sinclair, Jacqueline F: 5 articles (12/2007 - 04/2002)
10.	Sinclair, Peter R: 5 articles (12/2007 - 04/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Porphyria Cutanea Tarda:

1.	Chloroquine (Aralen)FDA LinkGeneric 11/01/1987 - "Considering the immediate effectiveness, good compliance, safety, lasting remission and low rate of relapse with prompt response on further treatment, high-dose, short-course treatment with chloroquine should be considered as the treatment of choice in porphyria cutanea tarda." 05/01/2002 - "Porphyria cutanea tarda can be effectively treated by phlebotomy or low dose chloroquine. " 10/21/1996 - "We determined whether chloroquine, which is effective in treating porphyria cutanea tarda, would mobilize an administered porphyrin from tissues and enhance its excretion. " 01/01/1984 - "The remission of porphyria cutanea tarda into a subclinical phase occurred after chloroquine therapy. " 02/01/1978 - "Treatment of a patient with porphyria cutanea tarda with low dose chloroquine improved the clinical and biochemical abnormalities in association with a reduction in photosensitivity as shown by changes in the action spectrum."
2.	IronIBA 04/01/2002 - "After the diagnosis, the patient underwent an aggressive series of therapeutic phlebotomies to reduce iron levels, and gradually the cutaneous manifestations of porphyria cutanea tarda improved." 01/01/2008 - "This study shows that increased liver iron content is not a constant finding in patients with porphyria cutanea tarda, especially in women, and that it is not a prerequisite for the efficiency of phlebotomy." 04/01/2002 - "Laboratory studies supported a diagnosis of porphyria cutanea tarda, complicated by the presence of both the C282Y and H63D mutations in the HFE gene, with susequent iron over-load. " 06/01/1990 - "Iron studies in the patient and immediate family members indicated that the proband was homozygous for haemochromatosis, but subsequent investigations revealed that porphyria cutanea tarda was responsible for her signs and symptoms. " 03/10/1977 - "Studies on 10 patients with porphyria cutanea tarda indicate that elevated iron levels are correlated with decreased latent and normal total iron binding capacity in the serum. "
3.	Hydroxychloroquine (Plaquenil)FDA LinkGeneric 05/01/1998 - "Childhood-onset porphyria cutanea tarda: successful therapy with low-dose hydroxychloroquine (Plaquenil)." 12/01/2012 - "Low-dose hydroxychloroquine is as effective as phlebotomy in treatment of patients with porphyria cutanea tarda." 04/01/1992 - "High-dose hydroxychloroquine is rarely used in the treatment of porphyria cutanea tarda (PCT). " 04/01/1992 - "High-dose hydroxychloroquine treatment of porphyria cutanea tarda." 05/01/1983 - "Hydroxychloroquine and phlebotomy were compared in the treatment of porphyria cutanea tarda (PCT). "
4.	Cimetidine (Biomet)FDA LinkGeneric 07/01/2010 - "Effective treatment for porphyria cutanea tarda with oral cimetidine." 09/01/1996 - "The efficacy of the H2 receptor antagonist, cimetidine, in the treatment of a patient with porphyria cutanea tarda (PCT) was evaluated. " 09/01/1996 - "Cimetidine in the treatment of porphyria cutanea tarda." 06/01/1995 - "Cimetidine, a H2-receptor antagonist, may be useful in the treatment of acute porphyric attack and in remission stage in neuroporphyrias, neurocutaneous porphyrias and cutaneous porphyrias such as porphyria cutanea tarda and protoporphyria."
5.	Uroporphyrinogen Decarboxylase (Uroporphyrinogen III Decarboxylase)IBA 01/01/2003 - "In this study 53 unrelated Danish patients with porphyria cutanea tarda were classified according to uroporphyrinogen decarboxylase and haemochromatosis gene mutations and the genotype related to the clinical and biochemical data. " 09/01/1989 - "Erythrocyte uroporphyrinogen decarboxylase activity in porphyria cutanea tarda: a study of 40 consecutive patients." 06/10/2013 - "Porphyria cutanea tarda (PCT) results from decreased activity of uroporphyrinogen decarboxylase (UROD) in the liver. " 04/01/2012 - "Porphyria cutanea tarda (PCT) is caused by decreased activity of uroporphyrinogen decarboxylase (UROD) in the liver. " 12/01/2010 - "Porphyria cutanea tarda (PCT) is a metabolic disorder that results in a decrease in uroporphyrinogen decarboxylase activity. "
6.	PorphyrinsIBA 06/01/1986 - "Five cases of porphyria cutanea tarda with mild cutaneous changes: evaluation of the efficacy of phlebotomy by the pattern analysis of urinary porphyrins." 01/01/2013 - "The high-performance liquid chromatography assessment revealed increased presence of porphyrins in blood and urine, thus confirming a diagnosis of porphyria cutanea tarda. " 03/01/2006 - "The clinical and laboratory findings were initially compatible with the suspicion of pseudoporphyria, but the finding of some frankly elevated levels of porphyrins in serum, urine and feces confirmed the diagnosis of porphyria cutanea tarda. " 07/01/2002 - "For each patient, histology and immunofluorescence was either consistent with the diagnosis of porphyria cutanea tarda or nonspecific, while serum, stool, and urine porphyrins did not support that diagnosis. " 01/15/2002 - "Excessive porphyrinuria of 3,128 nmol/24 h (normal < 165 nmol/24 h) with dominance of uro- and heptacarboxyporphyrin (75% of total porphyrins) indicated that the patient suffered from porphyria cutanea tarda. "
7.	anastrozole (Arimidex)FDA LinkGeneric 05/01/2007 - "A case of porphyria cutanea tarda resulting in digital amputation and improved by anastrazole." 08/01/2007 - "Remission of porphyria cutanea tarda after anastrozole treatment of breast cancer."
8.	Deferoxamine (Desferal)FDA LinkGeneric 05/01/1986 - "Twenty-five patients with overt clinical and biochemical findings of porphyria cutanea tarda took part in a study comparing intensive phlebotomy with slow subcutaneous desferrioxamine treatment. " 01/15/1965 - "[Porphyria cutanea tarda: study of the changes in porphyrin metabolism during desferrioxamine therapy]." 01/01/2003 - "[Successful treatment of haemodialysis-related porphyria cutanea tarda with deferoxamine]." 05/01/1990 - "Therefore, chronic intramuscular administration of desferrioxamine alone is ineffective in porphyria cutanea tarda." 05/01/1990 - "Four patients suffering from porphyria cutanea tarda received intramuscularly 10-15 g per month of desferrioxamine for periods ranging between 21 and 50 months. "
9.	Glutathione (Reduced Glutathione)IBA 05/01/1992 - "In order to evaluate the possible therapeutical effects of reduced glutathione in subjects affected by acquired or congenital heme biosynthesis alterations, two groups of subjects have been considered: the first, of 5 subjects with abnormal lead absorption, the second of 10 patients suffering from porphyria cutanea tarda (PCT). " 12/01/1999 - "A significant reduction was observed in retinol, alpha-, beta-carotene and red blood cell glutathione levels being more marked in porphyria cutanea tarda than in haemochromatosis patients. " 10/01/1979 - "In an in vitro experimental system, a study was also made of how the erythrocyte delta-aminolevulinic acid dehydratase activity varies on the action of the activators -SH and Zn2+. It was found that, compared to the healthy controls, the erythrocyte delta-aminolevulinic acid dehydratase activity of porphyria cutanea tarda patients is significantly decreased, but it is restored to the original activity level on the addition of -SH and Zn2+. Since there is a general -SH requirement of delta-aminolevulinic acid dehydratase, the most obvious explanation for the decrease of the activity in the case of the patients is the shift of the natural redox systems of the erythrocytes, and the decrease of the reduced glutathione/oxidized glutathione ratio."
10.	InterferonsIBA 03/01/1997 - "These results suggest that interferon may be beneficial for the treatment of porphyria cutanea tarda due to hepatitis C virus infection." 03/01/1997 - "We evaluated the efficacy of interferon in the treatment of a 61 year-old male patient with porphyria cutanea tarda associated with hepatitis C virus infection. " 11/01/2007 - "Porphyria cutanea tarda as rare cutaneous manifestation of hepatic metastases treated with interferon." 03/01/1997 - "Interferon treatment of porphyria cutanea tarda associated with chronic hepatitis type C." 02/01/1995 - "Normalization of urinary porphyrin level and disappearance of skin lesions after successful interferon therapy in a case of chronic hepatitis C complicated with porphyria cutanea tarda."

Therapies and Procedures

1.	Renal Dialysis (Hemodialysis) 06/01/2013 - "Porphyria cutanea tarda in a hemodialysis patient." 09/01/2010 - "Porphyria cutanea tarda in a chronic hemodialysis patient." 10/01/2008 - "Type I (sporadic) porphyria cutanea tarda in a hemodialysis patient: a case report." 01/01/2002 - "[Porphyria cutanea tarda, hemodialysis and HCV hepatopathy]." 06/01/2001 - "Cases of porphyria cutanea tarda (PCT) are occasionally reported in hemodialysis patients. "
2.	Amputation 05/01/2007 - "A case of porphyria cutanea tarda resulting in digital amputation and improved by anastrazole."
3.	Blood Transfusion (Blood Transfusions) 12/01/1996 - "In another 2 anti-HCV positive patients, porphyria cutanea tarda was preceded by blood transfusions. " 08/01/2001 - "We describe a case of porphyria cutanea tarda (PCT) induced by blood transfusion and oral iron supplementation in an 80-year-old white woman. " 08/01/2001 - "Porphyria cutanea tarda associated with an acute gastrointestinal bleed: the roles of supplemental iron and blood transfusion." 08/01/2012 - "To determine the efficacy and safety of deferasirox (an oral iron-chelating agent approved to reduce iron stores in patients with chronic iron overload due to blood transfusions) in a pilot trial for the treatment of patients with porphyria cutanea tarda (PCT), the most common of the porphyrias and often difficult to treat. "
4.	Transplants (Transplant) 06/01/2015 - "Sclerodermoid lesions in a patient with multiple transplants and porphyria cutanea tarda." 11/01/2012 - "Porphyria cutanea tarda in an HCV-positive liver transplant patient: a case report." 10/01/2010 - "Porphyria cutanea tarda (PCT) is rare in childhood and association with bone marrow transplant has occasionally been reported. " 01/01/2001 - "Porphyria cutanea tarda in a patient with post-transplant MDS." 01/01/1990 - "[Porphyria cutanea tarda in a patient undergoing bone marrow transplant. "
5.	Aftercare (After-Treatment) 10/01/1985 - "A significant linear correlation was found between serum total porphyrin concentration and 24-hour total urinary porphyrin excretion in 18 patients with porphyria cutanea tarda sampled at diagnosis and during and after treatment on 73 occasions. " 05/01/1972 - "[Liver changes in patients with porphyria cutanea tarda before and after treatment with repeated phlebotomies]." 11/01/2010 - "To evaluate skin changes in porphyria cutanea tarda using light microscopy and direct immunofluorescence before and after treatment with chloroquine. " 10/01/1988 - "Development of porphyria cutanea tarda after treatment with cyclophosphamide." 11/01/1987 - "Forty-six patients with porphyria cutanea tarda were observed for a mean period of 32 months (range 4-86) after treatment with high-dose, short-course chloroquine phosphate. "