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July 2016

Refractory Subcutaneous Sweet Syndrome Treated With Adalimumab

Author Affiliations
  • 1Duke University School of Medicine, Durham, North Carolina
  • 2Department of Pathology, Duke University School of Medicine, Durham, North Carolina
  • 3Department of Dermatology, Duke University School of Medicine, Durham, North Carolina

Copyright 2016 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.

JAMA Dermatol. 2016;152(7):842-844. doi:10.1001/jamadermatol.2016.0503

Subcutaneous Sweet syndrome (SSS) is a rare febrile neutrophilic dermatosis, distinct from classic Sweet syndrome. There is insufficient literature guidance regarding treatment options for SSS besides corticosteroids or effective alternatives if steroids fail to manage disease.

A man in his 50s presented with tender nodules on his buttocks and proximal extremities of 6 months’ duration and associated with fevers, joint pain, and swelling of the hands and feet. He had been previously hospitalized for fever, arthritis, and skin eruption. His erythrocyte sedimentation rate was 65 mm/h; C-reactive protein level, 14 mg/L; ferritin, 555 ng/mL; but findings were negative on an autoimmune workup (including serum protein electrophoresis [SPEP] and the anti–cyclic citrullinated peptide antibody test [anti-CCP], normal complement levels, and negative findings for antinuclear antibody, creatine kinase, antineutrophil cytoplasmic antibody, anti-Jo1, anti-Scl70, anti-dsDNA, antiribosomal P, antichromatin, anticentromere, and cryoglobulins). While infection was considered, findings from extensive testing were all negative (blood cultures, human immunodeficiency virus, hepatitis, Bartonella, Q fever, Rocky Mountain spotted fever, Lyme disease, and Epstein-Barr virus), and he failed to improve under treatment with multiple antibiotics.

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