Symptoms & signs

Schnitzler syndrome

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Symptoms & signs

Urticaria

The hallmark of Schnitzler syndrome is a chronic, recurrent urticarial rash, which is in most cases the first symptom to occur. In the beginning, pruritus is usually absent, but lesions may become mildly pruritic. The rash affects primarily the trunk and the extremities, sparing the palms, the soles, and the head and neck areas. The frequency of urticarial eruptions differs greatly among patients, ranging from daily to twice a year.

Periodic fever

Recurrent fever is the second most common symptom, affecting approximately 90% of patients. Like the urticarial eruptions, the frequency of febrile episodes differs greatly among patients, ranging from daily to twice a year. The episodes usually resolve within a few hours, but can persist for up to 24-48 hours. Peaks over 40ºC are common, though chills are rare. The fever and skin rash do not necessarily coincide.

Arthralgia or arthritis

About 80% of patients complain of relapsing arthralgias. Commonly, the large joints, including hips, knees, wrists and ankles, are affected, though there may be widespread involvement of joints. In some cases, arthritis is present, but no joint destruction has been reported.

Bone pain

Bone pain has been reported in about 45% of cases, and it typically affects the tibia and iliac bone. Some other ossa were incidentally affected, including the femur, forearm, spine and clavicle. On radiologic examination, bone densification is the most frequent finding.

General state

A high percentage of patients complains of malaise and fatigue. Weight loss is also common.

Findings on physical examination

The urticarial rash consists of annular erythematous and maculopapular lesions that are 0.5-3 cm in diameter and sometimes confluent. Lesions last 12-36 hours and resolve completely, while new ones appear daily.

Angioedema is a rare, but possible finding.

Lymphadenopathy, hepatomegaly and splenomegaly may be found as well.

Skin lesions in Schnitzler syndrome

For references see literature page

Last update/check: June 30, 2007