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Congenital erythropoietic porphyria

Congenital erythropoietic porphyria, or Günther disease, is a form of erythropoietic porphyria characterized by very severe and mutilating photodermatosis.

Splenomegaly

Abnormal increased size of the spleen.

Total: 1

(per page)

PMID (PMCID)
843024	FEMALE	Infant
	[Gunther's congenital erythropoietic porphyria in an 8-month old girl (author's transl)].
	Mascaro-Galy C, Mascaro JM, Albero F. Ann Dermatol Venereol. 1977;104(1):32-7.
	A typical case of congenital erythropoietic porphyria in a 8 month old girl is reported (photosensitive blistering syndrome, hypertrichosis; erythrodontia; fluorescence of urine, teeth, erythrocytes and myeloblasts; splenomegaly; anaemia).