mandibuloacral dysplasia with type B lipodystrophy

ORPHA: 90154

Available Treatments (0)

No treatments linked yet

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Clinical Presentation

Signs and symptoms associated with mandibuloacral dysplasia with type B lipodystrophy, sourced from HPO and Orphanet clinical annotations.

Narrow mouthAbnormality of the dentitionLarge fontanellesMicrognathiaConvex nasal ridgeProptosisDelayed pubertyInsulin resistanceAbnormality of the skeletal systemHyperpigmentation of the skinThin skinAbnormal fingertip morphologyAbnormal hair morphologyAlopeciaAcroosteolysis of distal phalanges (feet)OsteolysisHyperlipidemiaShort noseCalcinosisShort statureDermal atrophyProgeroid facial appearanceAplasia/Hypoplasia of the claviclesPrematurely aged appearanceNail dystrophyGeneralized lipodystrophyOsteolytic defects of the distal phalanges of the handShort distal phalanx of finger

Classification & Codes

Orphanet Code

ORPHA:90154
mandibuloacral dysplasia with type B lipodystrophy
OrphanetORPHA:90154
Treatments0 drug(s)
Symptoms on record28 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO