Porphyria Cutanea Tarda in a Patient With Agnogenic Myeloid Metaplasia
April 1984
in “
Archives of Dermatology
”
Porphyria cutanea tarda skin lesions sun-exposed areas increased fragility vesicles erosions pigmentation changes hypertrichosis sclerodermoid changes scarring alopecia calcification milia uroporphyrinogen decarboxylase deficiency autosomal-dominant inherited form sporadic form hepatocytes RBCs PCT skin fragility skin vesicles skin erosions skin pigmentation changes excessive hair growth skin thickening hair loss with scarring skin calcification skin milia URO-D deficiency inherited form red blood cells
TLDR Porphyria cutanea tarda causes skin issues due to an enzyme deficiency.
Porphyria cutanea tarda (PCT) is a syndrome marked by skin lesions in sun-exposed areas, characterized by increased fragility, vesicles, erosions, pigmentation changes, hypertrichosis, sclerodermoid changes with scarring alopecia, calcification, and milia. It results from a deficiency in uroporphyrinogen decarboxylase (URO-D), with two forms: an autosomal-dominant inherited form affecting both hepatocytes and RBCs, and a sporadic form affecting only hepatocytes. Symptoms typically appear after the fourth decade of life.
