clinical features

Last reviewed 01/2018

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.