mucolipidosis type IV

ICD-10: E75.1ORPHA: 578

Overview

Lysosomal storage disease

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 mucolipidosis type IV, sourced from HPO and Orphanet clinical annotations.

Everted lower lip vermilionMicrocephalyCoarse facial featuresStrabismusRetinopathyAbnormal electroretinogramPhotophobiaNystagmusMicrodontiaAtypical behaviorPalmoplantar keratodermaIntellectual disabilityAtaxiaHypotoniaGait disturbanceAbsent speechHyperreflexiaEEG abnormalityGenu recurvatumGanglioside accumulationBiparietal narrowingAbnormal nasal morphologyDevelopmental stagnationAbnormal retinal pigmentationCorneal opacityAplasia/Hypoplasia of the abdominal wall musculatureAbnormality of mucopolysaccharide metabolism

Classification & Codes

ICD-10 Code

E75.1

Orphanet Code

ORPHA:578
mucolipidosis type IV
ICD-10E75.1
OrphanetORPHA:578
Treatments0 drug(s)
Symptoms on record27 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO