Linear verrucous nevus syndrome

Summary

Linear verrucous nevus syndrome (MONDO:0016831) is a disease. A subtype of melanocytic nevus — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Phenotypes (HPO): 27

Clinical features

Signs & symptoms

Clinical features (HPO)

27 HPO clinical features (Orphanet curated; top 27 by frequency):

HPO ID	Term	Frequency
HP:0000256	Macrocephaly	Very frequent (80-99%)
HP:0000962	Hyperkeratosis	Very frequent (80-99%)
HP:0009592	Astrocytoma	Very frequent (80-99%)
HP:0100006	Neoplasm of the central nervous system	Very frequent (80-99%)
HP:0002209	Sparse scalp hair	Very frequent (80-99%)
HP:0012500	Verrucous papule	Very frequent (80-99%)
HP:0001250	Seizure	Frequent (30-79%)
HP:0001268	Mental deterioration	Frequent (30-79%)
HP:0000077	Abnormality of the kidney	Occasional (5-29%)
HP:0000481	Abnormal cornea morphology	Occasional (5-29%)
HP:0000486	Strabismus	Occasional (5-29%)
HP:0000518	Cataract	Occasional (5-29%)
HP:0000612	Iris coloboma	Occasional (5-29%)
HP:0000929	Abnormal skull morphology	Occasional (5-29%)
HP:0001305	Dandy-Walker malformation	Occasional (5-29%)
HP:0001770	Toe syndactyly	Occasional (5-29%)
HP:0001883	Talipes	Occasional (5-29%)
HP:0002119	Ventriculomegaly	Occasional (5-29%)
HP:0002650	Scoliosis	Occasional (5-29%)
HP:0002652	Skeletal dysplasia	Occasional (5-29%)
HP:0002816	Genu recurvatum	Occasional (5-29%)
HP:0004349	Reduced bone mineral density	Occasional (5-29%)
HP:0007370	Aplasia/Hypoplasia of the corpus callosum	Occasional (5-29%)
HP:0008060	Aplasia/Hypoplasia of the fovea	Occasional (5-29%)
HP:0010049	Short metacarpal	Occasional (5-29%)
HP:0000488	Retinopathy	Occasional (5-29%)
HP:0002148	Hypophosphatemia	Occasional (5-29%)

Identifiers

Disease identifiers

Field	Value
Canonical name	linear verrucous nevus syndrome
Mondo ID	MONDO:0016831
Orphanet	2611
UMLS	C5679838
MedGen	1806291
GARD	0003259
Is cancer (heuristic)	no

Also known as: linear hamartoma syndrome

Disease family

This is a subtype of melanocytic nevus. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › integumentary system disorder › integumentary system benign neoplasm › benign neoplasm of skin › melanocytic nevus › linear verrucous nevus syndrome

Related subtypes (27): conjunctival nevus, blue nevus, halo nevus, intradermal nevus, pigmented spindle cell nevus, nevus, epidermal, neurocutaneous melanocytosis, neutrophil actin dysfunction, CHILD syndrome, Becker nevus syndrome, CLOVES syndrome, nevus comedonicus syndrome, segmental outgrowth-lipomatosis-arteriovenous malformation-epidermal nevus syndrome, congenital panfollicular nevus, porokeratotic eccrine ostial and dermal duct nevus, hereditary mucosal leukokeratosis, nevus of Ota, nevus of Ito, phakomatosis pigmentokeratotica, PENS syndrome, Angora hair nevus, didymosis aplasticosebacea, scalp syndrome, Nevada syndrome, palpebral nevus, large congenital melanocytic nevus, benign melanocytic skin nevus

Subtypes (3): inflammatory linear verrucous epidermal nevus, verrucous nevus, acanthokeratolytic verrucous nevus

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 0.