Porphyria cutanea tarda (PCT) is the most prevalent form of porphyria worldwide. PCT is a dysfunction in uroporphyrinogen decarboxylase, a key enzyme in heme synthesis carried out in hepatocytes and erythrocytes in the bone marrow. PCT is characterized by photosensitivity with cutaneous signs of erosions and hyperpigmentation. The disease is exacerbated by alcohol consumption and estrogen supplementation. Furthermore, PCT is strongly associated with hepatitis C as a causative agent. The geriatric population is particularly vulnerable given their decrease in liver function, comorbidities, and elevated medication burden. Despite the complexity of the disease, the prognosis is favorable with appropriate management including the use of antimalarial medication, phlebotomy, and sun avoidance.

错误:搜索内容不能为空,请输入英文关键词
错误:关键词超出字数限制,请精简
高级检索

Porphyria Cutanea Tarda

  • Robert A. Norman,
  • Syed S. Ahmed

摘要

Porphyria cutanea tarda (PCT) is the most prevalent form of porphyria worldwide. PCT is a dysfunction in uroporphyrinogen decarboxylase, a key enzyme in heme synthesis carried out in hepatocytes and erythrocytes in the bone marrow. PCT is characterized by photosensitivity with cutaneous signs of erosions and hyperpigmentation. The disease is exacerbated by alcohol consumption and estrogen supplementation. Furthermore, PCT is strongly associated with hepatitis C as a causative agent. The geriatric population is particularly vulnerable given their decrease in liver function, comorbidities, and elevated medication burden. Despite the complexity of the disease, the prognosis is favorable with appropriate management including the use of antimalarial medication, phlebotomy, and sun avoidance.