lipoid proteinosis

ICD-10: E78.8MeSH: D008065ORPHA: 530

Overview

rare recessive genetic disorder

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 lipoid proteinosis, sourced from HPO and Orphanet clinical annotations.

Abnormality of the gingivaThick lower lip vermilionTongue nodulesAcneThickened skinSubcutaneous noduleHoarse voiceAbnormal blistering of the skinAbnormal oral mucosa morphologyScarringPapulePustuleMicroglossiaHigh palateHyperkeratosisDystoniaDysphagiaRecurrent respiratory infectionsAlopecia of scalpVerrucaeSeizureCerebral calcificationNasal polyposis

Classification & Codes

ICD-10 Code

E78.8

MeSH Code

D008065

Orphanet Code

ORPHA:530
lipoid proteinosis
ICD-10E78.8
MeSHD008065
OrphanetORPHA:530
Treatments0 drug(s)
Symptoms on record23 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO