Elsevier

The Lancet

Volume 344, Issue 8927, 1 October 1994, Pages 958-959
The Lancet

Letters to the Editor
Recessive inheritance of erythropoietic protoporphyria with liver failure

https://doi.org/10.1016/S0140-6736(94)92314-0Get rights and content

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    In clinical terms, these patients have the same dermatologic characteristics as the heterozygous patients. However, the risk of developing severe liver disease and liver failure requiring transplantation is much higher; close monitoring of liver function and protoporphyrin concentrations in plasma and feces is therefore recommended (Fig. 4).20 Recently, Holme et al.21 described a subgroup of 9 patients with recessive EPP (rEPP) with a characteristic palmar keratoderma.

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