Familial expansile osteolysis

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Familial expansile osteolysis

ORPHA:85195Disease

Also called Hereditary expansile polyostotic osteolytic dysplasia · McCabe disease

What it is

A rare primary bone dysplasia characterized by abnormal bone metabolism with bone pain, deformity, pathological fractures, early conductive hearing loss, and dental abnormalities. Focal bone lesions are typically found in the appendicular skeleton and consist of progressively expanding lytic areas, while generalized disordered bone modeling and altered trabecular pattern are the result of the multifocal, progressive nature of the disease. Age of onset is variable, mode of inheritance is autosomal dominant.

Key facts

Age of onset
Adolescent, Adult, Childhood
Inheritance
Autosomal dominant
Classified as
Disease

Gene

TNFRSF11ADisease-causing germline mutation(s)

ICD-10 codes

M89.5filed under a broader ICD-10 category — shared with 8 other rare diseases

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.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 9168MESH C536335MONDO 0008275OMIM 174810UMLS C0432292

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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