autoinflammatory disease, multisystem, with immune dysregulation, X-linked

Findings

No curated finding names autoinflammatory disease, multisystem, with immune dysregulation, X-linked yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.

Definition

Inheritance

X-linked recessive inheritance

Onset and course

Congenital onset · Infantile onset · Juvenile onset · Childhood onset

HPO, annotations 2026-09-02

What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.

Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.

Where it sits

A kind of

• autoinflammatory syndrome
• hereditary disorder of connective tissue

Narrower terms (2)

• early-onset autoimmune disorder due to DOCK11 partial deficiency
• early-onset immune dysregulation due to DOCK11 complete deficiency