Microscopic polyarteritis presenting with skin necrosis in a patient with sickle-cell disease.

A 20-year-old Caribbean woman with sickle cell anaemia was admitted with a 4-day history of fever and a painful swollen right ankle. She rapidly developed skin necrosis. The differential diagnosis is discussed. This case illustrates the difficulty in identifying the cause of cutaneous necrosis in an acutely ill patient. In our patient, histopathology implicated a vasculitic process, which was subsequently identified as a manifestation of microscopic polyarteritis.

Congenital erythropoietic porphyria: dilemmas in present day management.

Congenital erythropoietic porphyria is a rare autosomal recessive disorder of haem biosynthesis caused by a deficiency of uroporphyrinogen III synthetase. There is resultant accumulation and hyperexcretion of porphyrinogens of the isomer I variety. These are converted by spontaneous oxidation into their corresponding photoactive porphyrins leading to photodamage. Accumulation of porphyrins results in haemolysis and extensive photosensitivity. The consequences of chronic haemolysis are splenomegaly, reactive erythroid hyperplasia, erythrodontia, bone fragility, extreme photosensitivity and photomutilation. We present a 35-year-old man who has the severe infantile form and illustrates the haematological and photodestructive complications despite attempts at treatment with hypertransfusion, oral charcoal therapy and beta-carotene. Allogenic bone marrow transplantation has been considered but because of the high associated mortality this procedure has been discounted at present in the management of our patient.