Definition
NLRP1-associated auto-inflammation with arthritis and dyskeratosis (NAIAD) is a very rare, genetic auto-inflammatory disease detected in childhood. NAIAD is considered among them periodic fever syndromes / autoinflammatory diseases.
Symptoms
- Fever
- CRP is elevated
- Arthritis (may resemble Juvenile arthritis)
- Skin symptoms with dandruff
- Follicular hyperkeratosis
- Vitamin A deficiency may occur at the same time
- Painful eyes with photophobia
- Corneal dyskeratosis
- X-ray examinations can show skeletal changes in epiphyses at the knees
Treatment
- Arthritis can be treated with joint punctures
- The skin can respond well to acitretin
- Systemic inflammation can be treated with IL-1 inhibitors (Anakinra / kineret), Canakinumab / Ilaris)
Literature
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