syndromic recessive X-linked ichthyosis

ORPHA: 281090

Overview

Syndromic recessive X-linked ichthyosis (RXLI) refers to the cases of RXLI (see this term) that are associated with extracutaneous manifestations as part of a syndrome

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 syndromic recessive X-linked ichthyosis, sourced from HPO and Orphanet clinical annotations.

Intellectual disabilityGlobal developmental delayAphasiaAttention deficit hyperactivity disorderCorneal opacityCryptorchidismRenal insufficiencyUnilateral renal agenesisHypogonadismAutismSeizureLissencephalyAcute leukemiaAbnormal stomach morphologyAbnormal abdominal wall morphologyShort statureAbdominal wall defectTesticular seminomaHyperkeratosisHypohidrosisIchthyosis

Classification & Codes

Orphanet Code

ORPHA:281090
syndromic recessive X-linked ichthyosis
OrphanetORPHA:281090
Treatments0 drug(s)
Symptoms on record21 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO