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Ichthyosis-intellectual disability-dwarfism-renal impairment syndrome

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Disease definition

A rare multiple congenital anomalies syndrome characterized by nonbullous congenital ichthyosis (skin lesions are predominantly detected on the back and extensor surfaces of the limbs, including the flexures; the face is not affected), moderate intellectual disability, dwarfism and renal impairment. Moderate hirsutism may also be present. No significant facial dysmorphism has been reported. There have been no further descriptions in the literature since 1975.

ORPHA:2278

Classification level: Disorder

Synonym(s):
  • Passwell-Goodman-Siprkowski syndrome

Source: PubMed ID 1149323

Prevalence: <1 / 1 000 000

Inheritance: -

Age of onset: Neonatal

ICD-10: Q87.1

OMIM: 242530

UMLS: C4518580

MeSH: C536274

GARD: 4641

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2018.pdf) - Orphanet Urgences
Clinical practice guidelines
Disease review articles
Clinical genetics review
English (2013.pdf) - Eur J Hum Genet
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