linear nevus sebaceous syndrome
Findings
No curated finding names linear nevus sebaceous syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Linear nevus sebaceous syndrome (LNSS) is characterized by the association of a large sebaceous nevus, usually appearing on the face or on the scalp, with a broad spectrum of abnormalities that may affect every organ system, including the central nervous system (brain neoplasms, hemimegalencephaly and lateral ventricle enlargement).
Definition from the Mondo Disease Ontology (MONDO:0008097), read 2026-09-29. CC BY 4.0.
Features
31 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Adenoma sebaceumHPOHP:0009720
- Very frequent (80% to 99% of cases)
- AlopeciaHPOHP:0001596
- Very frequent (80% to 99% of cases)
- Aplasia/Hypoplasia of the cerebellumHPOHP:0007360
- Very frequent (80% to 99% of cases)
- Asymmetric growthHPOHP:0100555
- Very frequent (80% to 99% of cases)
- Biparietal narrowingHPOHP:0004422
- Very frequent (80% to 99% of cases)
- Cavernous hemangiomaHPOHP:0001048
- Very frequent (80% to 99% of cases)
- Diminished deep tendon reflex
Show the remaining 19
- Intellectual disabilityHPOHP:0001249
- Very frequent (80% to 99% of cases)
- Iris colobomaHPOHP:0000612
- Very frequent (80% to 99% of cases)
- Melanocytic nevusHPOHP:0000995
- Very frequent (80% to 99% of cases)
- MicrophthalmiaHPOHP:0000568
- Very frequent (80% to 99% of cases)
- Prominent occiputHPOHP:0000269
- Very frequent (80% to 99% of cases)
- SeizureHPOHP:0001250
- Very frequent (80% to 99% of cases)
Genes
1 gene
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- KRASHGNC:6407
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2021
Where it sits
Other names
9 names
Resolves to: linear nevus sebaceous syndrome
- Also called
- linear sebaceous NevusNevus sebaceous of JadassohnNevus sebaceus of JadassohnNevus Sebaceus Syndromeorganoid Nevusorganoid nevus syndromeSchimmelpenning syndromeSchimmelpenning-Feuerstein-Mims syndrome, somatic mosaicSolomon syndrome