Schnitlzler syndrome (SchS) is a rare auto-inflammatory disorder characterised by a chronic urticarial rash (hives), and signs and symptoms of systemic inflammation - including fever, bone pain, joint pain, muscle pain, headache, fatigue, and loss of weight. It was first described in 1972.

The cause is unknown.

Partly because of its rareness, it is currently believed to be under-diagnosed - possibly by a factor of 3.

During the past 42 years, SchS has evolved from an elusive little-known disorder to an autoinflammatory disorder that is recognized by increasing numbers of dermatologists, rheumatologists, allergologists, hematologists and other specialists.

Source : Clin Transl Allergy. 2014; 4: 41.