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Osteosclerosis-ichthyosis-premature ovarian failure syndrome

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Disease definition

A rare genetic disease characterized by sclerosing dysplasia affecting the diaphyseal and metaphyseal regions of the long bones, as well as the skull and metacarpals, in association with skin changes like those seen in ichthyosis vulgaris and premature ovarian failure with bilateral hypoplasia of the ovaries. Patients present in adulthood, primarily with swelling of the extremities and occasional mild pain in the legs.

ORPHA:75325

Classification level: Disorder

Synonym(s):
  • Sclerosing dysplasia of bone-ichthyosis-premature ovarian failure syndrome

Source: PubMed ID 15995452

Prevalence: <1 / 1 000 000

Inheritance: Unknown

Age of onset: Adolescent, Adult

ICD-10: M85.8

ICD-11: LD2F.1Y

OMIM: 609993

UMLS: C4302815

MeSH: C536064

GARD: 9904

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
Genetic testing
Guidance for genetic testing
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