Zinc-responsive necrolytic acral erythema

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Zinc-responsive necrolytic acral erythema

ORPHA:439196Particular clinical situation in a disease or syndrome

Also called Necrolytic acral erythema · NAE

What it is

A rare skin disease characterized initially by erythematous patches with superficial necrosis, at later stages by hyperkeratotic plaques with a rim of dusky erythema, of exclusively acral distribution with a predilection for the lower extremities, and universal association with hepatitis C virus infection. Patients typically experience pruritus or pain. Serum zinc levels may or may not be decreased, although oral zinc supplementation often improves the condition.

Key facts

Age of onset
All ages
Inheritance
Not applicable
Classified as
Particular clinical situation in a disease or syndrome

ICD-10 codes

L53.8filed under a broader ICD-10 category — shared with 1 other rare disease

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Cross-references

MEDDRA 10084061MONDO 0018586UMLS C4546437

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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