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Ichthyosis-oral and digital anomalies syndrome

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Disease definition

A rare genetic multiple congenital anomalies/dysmorphic syndrome characterized by ichthyosis, skeletal and digital anomalies (including flexion deformities of the large joints, proximal interphalangeal joints, absent distal flexion crease of fingers, tapering fingers, hypoplastic toes and nails, dislocatable ankles) and facial dysmorphism (including short forehead, epicanthic folds, small mouth with thin upper lip, midline lower lip groove, and fullness of the lateral eyelids, cleft palate can also be present). Additional clinical features may include developmental delay, generalized hypotonia, feeding difficulties, recurrent chest and gastrointestinal infections. There have been no further descriptions in the literature since 1989.

ORPHA:2272

Classification level: Disorder

Synonym(s):
  • Clayton Smith-Donnai syndrome

Source: PubMed ID 2732996

Prevalence: <1 / 1 000 000

Inheritance: Autosomal recessive

Age of onset: Infancy, Neonatal

ICD-10: Q87.8

ICD-11: LD27.2

OMIM: 258840

UMLS: C4518538

GARD: 2960

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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