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Linear nevus sebaceus syndrome
1 OMIM reference -
2 associated genes
76 connected diseases
31 signs/symptoms
Disease Type of connection
Costello syndrome
Noonan syndrome
Pilocytic astrocytoma
Hereditary nonpolyposis colon cancer
Cardiofaciocutaneous syndrome
Juvenile myelomonocytic leukemia
Familial pancreatic carcinoma
Phakomatosis pigmentokeratotica
LEOPARD syndrome
Noonan syndrome-like disorder with loose anagen hair
CLOVE syndrome
Cowden syndrome
Hemimegalencephaly
Macrodactyly of fingers, unilateral
Megalencephaly-capillary malformation-polymicrogyria syndrome
Segmental progressive overgrowth syndrome with fibroadipose hyperplasia
Split hand-split foot malformation
Follicular lymphoma
Intravascular large B-cell lymphoma
Capillary malformation - arteriovenous malformation
Hereditary gingival fibromatosis
Parkes Weber syndrome
Autosomal agammaglobulinemia
SHORT syndrome
Catecholaminergic polymorphic ventricular tachycardia
Craniopharyngioma
Hairy cell leukemia
Hashimoto-Pritzker syndrome
Hereditary spherocytosis
Joubert syndrome with orofaciodigital defect
Autosomal recessive nonsyndromic sensorineural deafness type DFNB
Chudley-McCullough syndrome
17q11 microdeletion syndrome
17q11.2 microduplication syndrome
Neurofibromatosis type 1 due to NF1mutation or intragenic deletion
Watson syndrome
Hyperinsulinism due to INSR deficiency
Insulin-resistance syndrome type A
Leprechaunism
Rabson-Mendenhall syndrome
Berardinelli-Seip congenital lipodystrophy
Diffuse cutaneous systemic sclerosis
Heritable pulmonary arterial hypertension
Limited cutaneous systemic sclerosis
Familial idiopathic steroid-resistant nephrotic syndrome with diffuse mesangial sclerosis
Familial idiopathic steroid-resistant nephrotic syndrome with focal segmental hyalinosis
Adrenocortical carcinoma
Autosomal dominant hypohidrotic ectodermal dysplasia
Autosomal dominant secondary polycythemia
B-cell chronic lymphocytic leukemia
Distal 22q11.2 microdeletion syndrome
Essential thrombocythemia
Giant cell glioblastoma
Gliosarcoma
Hypotrichosis simplex
Li-Fraumeni syndrome
Megalencephaly - polymicrogyria - postaxial polydactyly - hydrocephalus
Noonan syndrome-like disorder with juvenile myelomonocytic leukemia
Papilloma of choroid plexus
Precursor B-cell acute lymphoblastic leukemia
Autosomal dominant nonsyndromic intellectual deficit
Early-onset autosomal dominant Alzheimer disease
Hereditary cerebral hemorrhage with amyloidosis, Arctic type
Hereditary cerebral hemorrhage with amyloidosis, Dutch type
Hereditary cerebral hemorrhage with amyloidosis, Flemish type
Hereditary cerebral hemorrhage with amyloidosis, Iowa type
Hereditary cerebral hemorrhage with amyloidosis, Italian type
Hereditary cerebral hemorrhage with amyloidosis, Piedmont type
Renal tubular dysgenesis of genetic origin
Bilateral striopallidodentate calcinosis
Dermatofibrosarcoma protuberans
Familial multiple meningioma
Pediatric systemic lupus erythematosus
Autosomal dominant spastic paraplegia type 13
Papillary or follicular thyroid carcinoma
Pelizaeus-Merzbacher-like due to HSPD1 mutation
Synonym(s):
- Nevus sebaceus of Jadassohn
- Nevus sebaceus syndrome
- Organoid nevus syndrome
- Schimmelpenning syndrome
- Solomon syndrome
Classification (Orphanet):
- Rare developmental defect during embryogenesis
- Rare eye disease
- Rare genetic disease
- Rare oncologic disease
- Rare skin disease
Classification (ICD10):
- Congenital malformations, deformations and chromosomal abnormalities -
Epidemiological data:
Class of prevalence: unknown
Average age onset: childhood
Average age of death: any age
Type of inheritance: sporadic
External references:
1 OMIM reference -
No MeSH references
Gene symbol UniProt reference OMIM reference
HRAS P01112190020
KRAS P01116190070
Very frequent
- Adenoma sebaceum
- Anophthalmos / anophthalmia / microphthalmos / microphthalmia
- Areflexia / hyporeflexia
- Asymmetry of the body / hemiatrophy / hemihyperthrophy
- Autosomal dominant inheritance
- Cavernous / tuberous hemangioma
- Cerebellum / cerebellar vermis anomaly / agenesis / hypoplasia
- Coloboma of iris
- Dilated cerebral ventricles without hydrocephaly
- EEG anomalies
- Frontal bossing / prominent forehead
- Genu recurvatum
- Hypereflexia
- Hypotonia
- Hypotrichosis / atrichia / atrichiasis / scalp hairlessness
- Intellectual deficit / mental / psychomotor retardation / learning disability
- Narrow forehead
- Pigmented naevi / naevus pigmentosus / lentigo
- Prominent occiput / occipital bossing
- Seizures / epilepsy / absences / spasms / status epilepticus
- Telecanthus / canthal dystopy
- Vertebral segmentation anomaly / hemivertebrae

Frequent
- Anomalies of eyes and vision
- Facial structural asymmetry / facial hemiatrophy / facial hemihypertrophy
- Irregular / in bands / reticular skin hyperpigmentation
- Plagiocephaly
- Porencephaly

Occasional
- Corpus callosum / septum pellucidum total / partial agenesis
- Dandy-Walker anomaly
- Failure to thrive / difficulties for feeding in infancy / growth delay
- Intracranial / cerebral calcifications