Clinical features

Acute skin reactions may develop during direct sun exposure or shortly afterwards:

• burning, stinging and itching in sun-exposed skin:
  • especially the face and hands
  • skin lesions may be erythematous, oedematous or urticarial
  • rarely, there may be purpura or bullae on the nose and hands

Chronic skin manifestations include:

• small, atrophic waxy scars over nose and dorsa of hands where the skin lesions have resolved
• "aged" knuckles and fingers where the skin has thickened, wrinkled and become waxy
• superficial scarring of the nose, face and back of the neck

Other features include:

• haemolytic anaemia
• splenomegaly
• cholelithiasis
• hepatic failure
• increased amounts of protoporphyrin in erythrocytes, and usually, the faeces.