Epidermal nevus syndrome

ORPHA: 35125

Overview

a rare neurocutaneous syndrome

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with Epidermal nevus syndrome, sourced from HPO and Orphanet clinical annotations.

NevusHyperpigmentation of the skinHypopigmentation of the skinSpinal cord compressionSpinal canal stenosisAtrophy of the spinal cordProgressive spastic paraparesisPainAstigmatismVisual impairmentDelayed speech and language developmentGlobal developmental delayHypertoniaAreflexiaThoracolumbar scoliosisBabinski signWeakness of long finger extensor musclesSpinal cord tumorLipomaAbnormal brain morphologyPolycystic kidney dysplasiaOsteopeniaAbnormal facial shapeRhabdomyosarcomaAortic aneurysmDecreased circulating vitamin D concentration

Classification & Codes

Orphanet Code

ORPHA:35125
Epidermal nevus syndrome
OrphanetORPHA:35125
Treatments0 drug(s)
Symptoms on record26 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO