Conditions / Immune
CINCA Syndrome
info · Immune · ICD-10: E85.0
An autoimmune disease characterized by neonatal onset of cutaneous symptoms, chronic meningitis, and joint manifestations with recurrent fever and inflammation that has_material_basis_in heterozygous mutation in the NLRP3 gene on chromosome 1q.
Signs and symptoms
- Anemia
- Lymphadenopathy
- Elevated erythrocyte sedimentation rate
- Elevated circulating C-reactive protein concentration
- Skin rash
- Hepatosplenomegaly
- Increased total leukocyte count
- Recurrent fever
- Frontal bossing
- Papilledema
Also known as: IOMID syndrome; NOMID syndrome; Prieur-Griscelli syndrome; chronic infantile neurological cutaneous articular syndrome; chronic neurologic cutaneous and articular syndrome