hereditary lymphedema II

ORPHA: 90186

Overview

hereditary lymphedema characterized by onset around puberty of chronic lymphedema particularly in the lower limbs with an apparent autosomal dominant pattern of inheritance

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 hereditary lymphedema II, sourced from HPO and Orphanet clinical annotations.

LymphedemaAtypical scarring of skinRecurrent skin infectionsLymph node hypoplasiaAbsence of lymph node germinal centerPredominantly lower limb lymphedemaRecurrent bacterial skin infectionsPedal edemaSkin dimpleCobblestone-like hyperkeratosisCellulitisSkin erosionFacial edemaPleural effusionVaricose veinsEdema of the dorsum of handsLaryngeal edemaPeripheral edemaPeriorbital edemaSkin ulcerAngiosarcoma

Classification & Codes

Orphanet Code

ORPHA:90186
hereditary lymphedema II
OrphanetORPHA:90186
Treatments0 drug(s)
Symptoms on record21 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO