Porphyria Cutanea Tarda



Porphyria Cutanea Tarda


Michael W. Peterson, DO

Cary Chisholm, MD

Clay J. Cockerell, MD










Skin manifestations of porphyria are best characterized by blister formation image.






Histologically PCT shows a subepidermal cell-poor blister with festooning of dermal papillae and caterpillar bodies image. Prominent solar elastosis image is also seen in the dermis.


TERMINOLOGY


Abbreviations



  • Porphyria cutanea tarda (PCT)


Definitions



  • Group of heterogeneous metabolic disorders caused by errors in heme synthesis



    • Variants of porphyria besides PCT include



      • Variegate porphyria (VP)


      • Hereditary coproporphyria (HCP)


      • Erythropoietic protoporphyria (EPP)


      • Acute intermittent porphyria (no skin findings) (AIP)


      • Congenital erythropoietic porphyria (CEP)


      • Hepatoerythropoietic porphyria (HEP)


    • PCT is most common variant; caused by deficiency in enzyme uroporphyrinogen decarboxylase

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Porphyria Cutanea Tarda
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