Coproporphyria congenita erythropoeticaE80.2
Synonym(s)
Coproporphyria erythropoietic; Erythropoietic coproporphyria
DefinitionThis section has been translated automatically.
Very rare, probably autosomal-dominantly inherited disorder of the porphyrin metabolism.
ManifestationThis section has been translated automatically.
Early childhood.
Clinical featuresThis section has been translated automatically.
Integument: Reddish-livid, succulent, itchy infiltrates or persistent, livid erythema and blistering of the back of the hand after sun exposure. S.u. Porphyria cutanea tarda.
LaboratoryThis section has been translated automatically.
Red-fluorescent erythrocytes in the blood smear (detection is particularly successful during the low light season). Increase of coproporphyrins III and protoporphyrins III in the erythrocytes.
Differential diagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
Acute treatment by internists. Prophylactic textile and chemical/physical light protection (e.g. Anthelios, see also light protection agent), if necessary reversal of the day-night rhythm.