Coproporphyria congenita erythropoetica - Altmeyers Encyclopedia

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.

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Dermatology

Lichen planus classic type

Non-contagious, subacute to chronic, markedly pruritic, usually self-limited (duration of disease between 1...

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Clinic
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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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Protoporphyria erythropoetica.

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.