dyskeratosis congenita

MeSH: D019871ORPHA: 1775

Overview

rare progressive congenital disorder with a highly variable phenotype

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 dyskeratosis congenita, sourced from HPO and Orphanet clinical annotations.

Abnormal morphology of female internal genitaliaAbnormal testis morphologyAbnormality of the dentitionHypoplasia of the maxillaHearing impairmentBlepharitisAbnormal eyelash morphologyCataractAbnormal eyebrow morphologyAbnormality of the pharynxHypodontiaCarious teethTaurodontiaPeriodontitisDiabetes mellitusOsteoporosisHyperhidrosisPalmoplantar keratodermaHypermelanotic maculeHypopigmented skin patchesAbnormal fingernail morphologyGlobal developmental delayCirrhosisHepatic failureIntrauterine growth retardationAlopeciaSplenomegalyThrombocytopeniaAbnormality of neutrophilsAnemiaAbnormality of coagulationMalabsorptionRecurrent respiratory infectionsPremature graying of hairHepatomegalyCerebral calcificationTracheoesophageal fistulaScoliosisNeoplasmLymphomaOral leukoplakiaRecurrent fracturesNeoplasm of the pancreasShort statureCellular immunodeficiencyBone marrow hypocellularityAplasia/Hypoplasia of the skinAbnormal blistering of the skinSparse hairNail dystrophyUrethral stenosisEsophageal stenosisAplastic/hypoplastic toenailAvascular necrosisWhite hairAnorectal anomalyMaculeTelangiectasia of the skinDisplacement of the urethral meatusCoarse metaphyseal trabecularizationSkin vesicleSkin ulcer

Classification & Codes

MeSH Code

D019871

Orphanet Code

ORPHA:1775
dyskeratosis congenita
MeSHD019871
OrphanetORPHA:1775
Treatments0 drug(s)
Symptoms on record62 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO