Linear nevus sebaceus syndrome

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Linear nevus sebaceus syndrome

ORPHA:2612Disease

Also called Nevus sebaceus of Jadassohn · Nevus sebaceus syndrome · Organoid nevus syndrome · Schimmelpenning syndrome · Solomon syndrome

What it is

A rare nevus syndrome characterized by the association of an nevus sebaceous with a broad spectrum of abnormalities that affect many organ systems, most commonly the eye, skeletal and central nervous system.

Key facts

Age of onset
Childhood, Infancy, Neonatal
Inheritance
Not applicable
Classified as
Disease

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Genes

HRASDisease-causing somatic mutation(s)
KRASDisease-causing somatic mutation(s)
NRASCandidate gene tested

ICD-10 codes

Q85.8filed under a broader ICD-10 category — shared with 16 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 10291MESH D054000MONDO 0008097OMIM 163200UMLS C4552097

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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