Adult-onset congenital erythropoietic porphyria (Günther's disease) presenting with thrombocytopenia.
Cutaneous signs of Günther's disease (congenital erythropoietic porphyria) developing 5 years after the onset of symptomatic thrombocytopenia are described in a 65-year-old man. Persistent thrombocytopenia unresponsive to corticosteroids and immunoglobulin necessitated a splenectomy.
Murphy
A
A
Regional Centre of Dermatology, Mater Hospital, Dublin, Eire.
Gibson
G
G
Elder
G H
GH
Otridge
B A
BA
Murphy
G M
GM
D000368:Aged; D006801:Humans; D008297:Male; D017092:Porphyria, Erythropoietic / Q000150:complications* / Q000601:surgery; D013156:Splenectomy; D013921:Thrombocytopenia / Q000209:etiology*