Coproporphyria congenita erythropoetica E80.2

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Coproporphyria erythropoietic; Erythropoietic coproporphyria

Definition
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Very rare, probably autosomal-dominantly inherited disorder of the porphyrin metabolism.

Manifestation
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Early childhood.

Clinical features
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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.

Laboratory
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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 diagnosis
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Therapy
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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.

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020