Necrobiotic xanthogranuloma

Orpha code: 158011OMIM code:

Definition

Necrobiotic xanthogranuloma is a rare, chronic and progressive, non-Langerhans cell histiocytosis disease typically characterized by multiple, indurated, asymptomatic to pruritic, yellow-orange plaques or nodules that tend to ulcerate and are usually located in the periorbital area, trunk and/or extremities. Strong association with paraproteinemia and/or malignant lymphoproliferative disease has been reported.

Disease data
Classification

Disease

ORPHA code
158011
OMIM code
-
ICD10 code
D76.3
ICD11 code
EE8Y

No additional description.

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