trichorhinophalangeal syndrome type I

MeSH: C536820ORPHA: 77258

Overview

autosomal dominant disease that is characterized by short stature, sparse hair, a bulbous nasal tip and cone-shaped epiphyses (the growing ends of bones), as well as severe generalized shortening of all finger and toe bones (brachydactyly)

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 trichorhinophalangeal syndrome type I, sourced from HPO and Orphanet clinical annotations.

Triangular faceLong philtrumMicrognathiaMacrotiaProtruding earBulbous noseSparse eyelashesFrontal bossingClinodactyly of the 5th fingerShort statureSparse hairShort distal phalanx of fingerShort metacarpalCone-shaped epiphysisShort metatarsalLong upper lipShortening of all phalanges of fingersSparse eyebrowAbnormality of the dentitionHigh palatePectus carinatumHypotoniaFragile nailsLeukonychiaScoliosisHyperlordosisAvascular necrosis of the capital femoral epiphysisSupernumerary toothCamptodactyly of finger

Classification & Codes

MeSH Code

C536820

Orphanet Code

ORPHA:77258
trichorhinophalangeal syndrome type I
MeSHC536820
OrphanetORPHA:77258
Treatments0 drug(s)
Symptoms on record29 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO