Sonozaki syndrome in the spotlight of imaging

Excerpt

Sonozaki syndrome (SS) also known as pustulotic arthro-osteitis (PAO) is a rare chronic inflammatory disease belonging to the group of spondyloarthritis (SpA), firstly described by Sonozaki [1]. Onset is frequently seen at age 30–40, with a similar prevalence between sexes. Sterile palmoplantar pustular lesions and anterior thoracic joint involvement (with 77% of manubrium sterni) are the most typical findings, although other joints can be involved (34% of axial, 32% of peripheral joints, and 13% of sacroiliitis). We present the case of a 30-year-old male with palmoplantar pustulosis (PPP) and painful swelling of the sternum, clavicles, and lower ribs. The patient had no family history of other rheumatic disorders. General physical examination resulted negative for clinical involvement of the spinal column, and of the sacroiliac and peripheral joints, with no signs or symptoms of infective disease. Laboratory findings showed mild increase of erythrocyte sedimentation rate (ESR) (50 mm/h) and C-reactive protein (CRP) (2.5 mg/dl, n.v. 0.5); other biochemical tests (blood count, serum electrolytes, creatinine, urea, uric acid, glucose, bilirubin, transaminases, creatine phosphokinase, LDH, alcaline phosphatase), protein levels with electrophoresis, and general tests of urine, immunoglobulins, and complement and anti-CCP were normal; rheumatoid factor (RA test and Waaler rose) and antinuclear antibodies (ANA) were negative. HLA typing resulted negative for aplotipo HLA-B51, 52, and 27. Skin biopsy confirmed the suspected PAO diagnosis. The evaluation of the patient at 6 and 12 months confirmed the complete recovery after therapy with 16 mg of metilprednisolone with descending sequences of dose for 2 months. …