Autoinflammation, Panniculitis, and Dermatosis Syndrome

**Semantic type:** Disease or Syndrome

**Definition:** An autosomal recessive condition caused by mutation(s) in the OTULIN gene, encoding ubiquitin thioesterase otulin. It is characterized by neonatal onset of recurrent fever, erythematous rash with painful nodules, painful joints, and lipodystrophy.

**Synonyms:** - AIPDS - ORAS - Otulin-Related Autoinflammatory Syndrome - Otulipenia