Revesz syndrome

MeSH: C538371ORPHA: 3088

Overview

dyskeratosis congenita that has material basis in an X-linked recessive mutation of TINF2 on chromosome 14q12

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 Revesz syndrome, sourced from HPO and Orphanet clinical annotations.

Bone marrow hypocellularityIntracranial calcificationMicrocephalyRetinal detachmentCerebellar hypoplasiaGrowth delayAplastic anemiaOral leukoplakiaFine, reticulate skin pigmentationSerous retinal detachmentSparse hairNail dystrophyNeurodevelopmental delaySeizureAtaxiaFailure to thrive in infancyDermal atrophy

Classification & Codes

MeSH Code

C538371

Orphanet Code

ORPHA:3088
Revesz syndrome
MeSHC538371
OrphanetORPHA:3088
Treatments0 drug(s)
Symptoms on record17 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO