primary hypergonadotropic hypogonadism-partial alopecia syndrome

ORPHA: 2232

Overview

This syndrome is characterized by primary hypergonadotropic hypogonadism and partial alopecia

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 primary hypergonadotropic hypogonadism-partial alopecia syndrome, sourced from HPO and Orphanet clinical annotations.

Hypergonadotropic hypogonadismAlopecia of scalpPrimary gonadal insufficiencyCryptorchidismThin upper lip vermilionAbnormal eyebrow morphologyPrimary amenorrheaInfertilityImpotenceDelayed pubertyIncreased circulating gonadotropin levelOsteopeniaOsteoporosisGrowth delayAlopeciaBreast hypoplasiaMarked delay in bone ageFlat occiputSparse facial hairAbsence of secondary sex characteristicsDecreased serum estradiolAgonadismAplasia/hypoplasia of the uterusAplasia of the ovaryStreak ovaryNon-obstructive azoospermiaDecreased serum testosterone concentrationMicrocephalyMild intellectual disabilitySparse pubic hairSkeletal dysplasiaKyphosisLumbar hyperlordosisSparse eyebrowHearing impairmentTriphalangeal thumbThenar muscle atrophyDecreased palmar creasesContracture of the proximal interphalangeal joint of the 5th fingerSmall hypothenar eminenceSmall pituitary gland

Classification & Codes

Orphanet Code

ORPHA:2232
primary hypergonadotropic hypogonadism-partial alopecia syndrome
OrphanetORPHA:2232
Treatments0 drug(s)
Symptoms on record41 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO