Abstract :
Background: The porphyria's are a rare group of metabolic disorders produced by acquired or hereditary deficiency of the enzyme UROD, fifth enzyme in the chain of production of the heme group, which results in an accumulation of photosensitive by products, such as uroporphyrinogen, which leads to the fragility and blistering of sun-exposed skin. Porphyria can manifest with neurovisceral and/or cutaneous symptoms, depending on the defective enzyme. Prevalence of porphyria's varies, from 1 in 500 to 1 in 50,000 people worldwide. PCT, the most common.
Case Presentation: A 59-year-old Indian man presented with multiple non healing ulcers on dorsum of right hand and scalp associated with photosensitivity and reddish discoloration of urine, and surrounding depigmentation with scarring. He was diagnosed as having PCT after clinical investigation and was treated symptomatically.
Conclusion: PCT is the most common of the seven porphyrin metabolism disorders. The misdiagnosis and mismanagement of this disease can have a significant impact on a patient's life and does management of PCT in our case is focused on the signs and symptoms and findings.
Keyword :
Porphyria cutanea tarda, Uroporphyrinogen decarboxylase