Lenz–Majewski syndrome

MeSH: C537115ORPHA: 2658

Overview

Lenz-Majewski hyperostotic dwarfism is an extremely rare syndrome associating dwarfism, characteristic facial appearance, cutis laxa and progressive bone sclerosis

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 Lenz–Majewski syndrome, sourced from HPO and Orphanet clinical annotations.

Joint hypermobilityLarge fontanellesMacrocephalyDelayed cranial suture closureMandibular prognathiaHypertelorismBroad foreheadMacrotiaChoanal atresiaAbnormal dental enamel morphologyAbnormal metaphysis morphologyBrachydactylyIntellectual disabilityGlobal developmental delaySpecific learning disabilityRedundant skinThickened calvariaDelayed skeletal maturationAbnormal cortical bone morphologySevere short statureShort palmCranial hyperostosisFacial hyperostosisFinger syndactylyAplastic claviclePrematurely aged appearanceAplasia/Hypoplasia of the skinIncreased bone mineral densityOsteopetrosisInguinal herniaCryptorchidismAbnormal penis morphologyEpispadiasHypospadiasWide mouthAbnormal nasolacrimal system morphologyAbnormal finger morphologyElbow ankylosisAbnormal metacarpal morphologyFacial palsyThick vermilion borderFemoral herniaHypogonadismCleft palateSubmucous cleft hard palateBifid uvulaHydrocephalusHypotoniaAgenesis of corpus callosumAbsent septum pellucidumLimitation of joint mobilityHypoplastic fingernailHyperconvex fingernailsScoliosisHigh, narrow palateKyphosisExternal genital hypoplasia

Classification & Codes

MeSH Code

C537115

Orphanet Code

ORPHA:2658
Lenz–Majewski syndrome
MeSHC537115
OrphanetORPHA:2658
Treatments0 drug(s)
Symptoms on record57 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO