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J R Soc Med. 1995 June; 88(6): 357P–358P.
PMCID: PMC1295249

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.

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Selected References

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  • Kramer S, Viljoen E, Meyer AM, Metz J. The anaemia of erythropoietic prophyria with the description of the disease in an elderly patient. Br J Haematol. 1965 Nov;11(6):666–675. [PubMed]
  • Pain RW, Welch FW, Woodroffe AJ, Handley DA, Lockwood WH. Erythropoietic uroporphyria of Gunther first presenting at 58 years with positive family studies. Br Med J. 1975 Sep 13;3(5984):621–623. [PMC free article] [PubMed]
  • Rank JM, Straka JG, Weimer MK, Bossenmaier I, Taddeini BL, Bloomer JR. Hematin therapy in late onset congenital erythropoietic porphyria. Br J Haematol. 1990 Aug;75(4):617–618. [PubMed]
  • MacDonald A, Nicholson D, Williams R. Late onset erythropoietic uroporphyria. Br J Dermatol. 1976 Jul;95 (Suppl 14):30–33. [PubMed]
  • Horiguchi Y, Horio T, Yamamoto M, Tanaka T, Seki Y, Imamura S. Late onset erythropoietic porphyria. Br J Dermatol. 1989 Aug;121(2):255–262. [PubMed]
  • Haining RG, Cowger ML, Shurtleff DB, Labbe RF. Congenital erythropoietic porphyria. I. Case report, special studies and therapy. Am J Med. 1968 Oct;45(4):624–637. [PubMed]

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