Schnitzler's syndrome

Abstract

We report on a case of Schnitzler's syndrome, focusing on pattern of bone involvement and its differential diagnosis.

Management of Schnitzler’s syndrome

“…Antihistamines are almost invariantly ineffective in treatment of the frequently nonpruritic urticarial rash of Schnitzler's syndrome [11][12][13][14][15]. This most likely reflects the fact that histamine is not implicated in the pathophysiology of skin lesions in this condition.…”

Schnitzler syndrome: clinical features and histopathology

“…9 If MRI is performed, this typically shows evidence of cortical thickening as well as medullary bone involvement in the absence of a tumor. 15,16 Sometimes, the abnormality is restricted to the medulla with T 2 hyperintensity.…”

“…In one series with 22 patients, a radiologic abnormality was found in 64% of the patients, 9 although in some series, the frequency of radiologic musculoskeletal findings was lower (40%). 14,15 The most common radiologic finding is bone sclerosis that most commonly affects the bone around the knee, with the femur somewhat more likely to be affected compared to the tibia. 15 The pattern of sclerosis varies from trabecular thickening to patchy or confluent osteosclerosis.…”

“…14,15 The most common radiologic finding is bone sclerosis that most commonly affects the bone around the knee, with the femur somewhat more likely to be affected compared to the tibia. 15 The pattern of sclerosis varies from trabecular thickening to patchy or confluent osteosclerosis. The sclerotic process always extends to involve the endosteum.…”

Schnitzler’s Syndrome: Monoclonal Gammopathy Associated with Chronic Urticaria

“…LPD consists mostly of Waldenström macroglobulinemia [3]; in addition, lymphoplasmacytic lymphoma [4], multiple myeloma, and marginal zone B lymphoma [5] have been described in sporadic cases. The evolution to AA amyloidosis was reported in 3 patients [6,7,8]. …”