Introduction

Porphyria cutanea tarda is the most common hepatic porphyria. It is due to an inherited defect in uroporphyrinogen decarboxylase or caused by hepatocyte damage e.g. alcohol, hepatitis C, oestrogen.

Clinical features

Features
  • classically presents with photosensitive rash with blistering and skin fragility on the face and dorsal aspect of hands (most common feature)
  • hypertrichosis
  • hyperpigmentation

Investigations

Investigations
  • urine: elevated uroporphyrinogen and pink fluorescence of urine under Wood's lamp

Management

Management
  • chloroquine
  • venesection