Porphyria

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porphyria cutanea tarda

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Porphyria

  1. 1. KURSK STATE MEDICAL UNIVERSITY DEPARTMENT OF SKIN AND VENEREAL DISEASES HEAD OF THE DEPT.-Prof. Silina L.V. TEACHER- E.I.Sirotkina Porphyria Cutanea Tarda Presented by : Aayupta Mohanty 4th year ,group 5 2013
  2. 2. Introduction  The porphyrias are caused by deficiencies of enzymes involved in heme biosynthesis which lead to blockade of the porphyrin pathway and subsequent accumulation of porphyrins and their precursors.
  3. 3. Introduction   Seven major types of porhyria are now recognized . They include acute and non-acute forms.
  4. 4. Classification of porphyrias
  5. 5. Introduction   Cutaneous features are not seen in acute intermittent porphyria (AIP) or the very rare aminolevulinic acid dehydratase (ALA-D) deficient porphyria. Erythropoietic protoporphyria and congenital erythropoietic porphyria are characterized by porphyrins produced mainly in the bone marrow. The reminder are primarily hepatic porhyrias.
  6. 6. Introduction  Excessive concentrations of porhyrins exposed to day-light generate free radicals, leading to cell membrane damage and cell death.
  7. 7. Introduction  The type of cellular damage depends on the solubility and tissue distribution of the porphyrins. Two main patterns of skin damage are seen in the porphyries: 1. accumulation of water soluble uro- and coproporphyrins leads to blistering. 2. accumulation of the lipophilic protoporphyrins leads to burning
  8. 8. PORPHYRIA CUTANEA TARDA :
  9. 9. Epidimiology      It is the most common porphyria. It may be acquired (type I) genetically inherited (typeII). or 60% of PCT patients are male, most of whom ingest excess alcohol. Women who develop PCT are often on estrogen-containing medications. Most patients are ≥ 40years, and 66% have evidence of iron overload.
  10. 10. Pathogenesis   Iron overload leads to reduce activity of the uroporphyrinogen decarboxylase enzyme which leads to elevated porphyrin levels, in particular uroporphyrins. Associated disorders :       Alcoholism. Hematochromatosis. HCV. HIV. HBV. CMV.
  11. 11. Pathogenesis    PCT presenting in a young adult should lead to consideration of HIV infection , alternatively familial PCT could be the explanation. familial PCT (typeII) accounts for 10-20 % of cases. It is inherited as an autosomal dominant trait. Most PCT is acquired (typeI) and multifactorial in origin.
  12. 12.   Diagnostic Considerations Other porphyrias, pseudoporphyrias, and photoaggravated bullous dermatoses can manifest with clinical features indistinguishable from those of porphyria cutanea tarda. Failure to obtain sufficient biochemical confirmation of the diagnosis can lead to inappropriate treatment of non–porphyria cutanea tarda disorders.  Differential Diagnoses  Epidermolysis Bullosa  Epidermolysis Bullosa Acquisita  Erythropoietic Porphyria  Hydroa Vacciniforme  Lupus Erythematosus, Bullous 
  13. 13. Investigation
  14. 14. Pathology  Subepidermal blister with minimal “cell-poor ” dermal inflammatory infiltrate.
  15. 15. Treatment

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