Treatment of porphyria cutanea tarda with chloroquine

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  1. Malder Moderator

    Treatment of porphyria cutanea tarda with chloroquine


    Porphyria Cutanea Tarda (PCT) is a rare disorder characterized by painful, blistering skin lesions that develop on sun-exposed skin (photosensitivity). Affected skin is fragile and may peel or blister after minor trauma. PCT is essentially an acquired disease, but some individuals have a genetic (autosomal dominant) deficiency of UROD that contributes to development of PCT.

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    High-dose chloroquine therapy for porphyria cutanea tarda is rarely used now because of its hepatic side-effects. The mechanisms of the effects and side-effects are poorly understood. We describe here effects, side-effects and long-term follow-up in 57 patients with a first-time diagnosis of porphyria cutanea tarda treated with 1–3. Hydroxychloroquine Plaquenil® is a 4-amino-quinoline antimalarial medication that is widely used to treat systemic lupus erythematosus SLE, rheumatoid arthritis, and related inflammatory and dermatological conditions. It is a hydroxylated version of chloroquine, with a similar mechanism of action. Porphyria cutanea tarda PCT is common in Ethiopia and invariably affects the liver. Treatment by abstension from alcohol and avoidance of direct sunlight often failed to achieve lasting improvement.

    Most individuals with the inherited enzyme deficiency remain latent and never have symptoms. These individuals are referred to as having "familial PCT".

    Treatment of porphyria cutanea tarda with chloroquine

    Porphyria cutanea tarda a case report Journal of Medical., Hydroxychloroquine DermNet NZ

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  5. Porphyria cutanea tarda PCT is a term encompassing a group of acquired and familial disorders in which activity of the heme synthetic enzyme uroporphyrinogen decarboxylase UROD is deficient. Approximately 80% of all cases of porphyria cutanea tarda are acquired; 20% are familial, although the ratio may vary among different geographic regi.

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    The skin lesions of pseudoporphyria closely resemble those seen in cutaneous forms of porphyria including porphyria cutanea tarda. Pseudoporphyria can occur at any age. Women are affected more often than men. Nov 18, 2017 Treatment of acute porphyria attacks focuses on providing rapid treatment of symptoms and preventing complications. Treatment may include Injections of hemin, a medication that is a form of heme, to limit the body's production of porphyrins; Intravenous sugar glucose, or sugar taken by mouth, if able, to maintain an adequate intake of carbohydrates Chloroquine treatment can induce porphyria attacks within the first couple of months of treatment due to the mass mobilization of porphyrins from the liver into the blood stream. Complete remission can be seen within 6–12 months as each dose of antimalarial can only remove a finite amount of porphyrins and there are generally decades of accumulation to be cleared.

     
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    Hydroxychloroquine Retinopathy Still Alive and Well - The. May 16, 2011 Hydroxychloroquine Plaquenil; HCQ has been an important and effective drug for the treatment of lupus erythematosus and related autoimmune and inflammatory diseases for half a century, although its potential to cause retinal damage continues to raise concern among rheumatologists and ophthalmologists.

    Hydroxychloroquine Plaquenil Toxicity and Recommendations.