7 research outputs found
Incomplete Schnitzler Syndrome
Schnitzler syndrome (SS) is a rare autoinflammatory disease that
presents with chronic urticaria and monoclonal immunoglobulin (Ig) M or G,
accompanied by fever, abnormal bone remodeling, skin biopsy with a neutrophilic
dermal infiltrate, leukocytosis, or elevated C-reactive protein. It is usually
refractory to antihistamines and immunosuppression. We present a case report
of clinical SS without monoclonal Ig with robust response to interleukin-1 inhibitor
anakinra. This suggests the possible existence of an incomplete form of
SS and underlines the risk of false negative diagnosis in individuals with such
“incomplete SS”