Pediatric Rheumatology Online Journal → July 2003 → Childhood Lupus→ Case Reports → Abstract #110

SWEET SYNDROME IN A CHILD WITH LUPUS AND SUBSEQUENT SJOGREN SYNDROME

J. M. Burnham,¹ R. Q. Cron.¹

¹Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA

INTRODUCTION: Sweet Syndrome is a rare dermatologic eruption that has been reported in adults with Lupus and Sjogren Syndrome. Also known as Acute Febrile Neutrophilic Dermatosis, Sweet Syndrome results in recurrent, often painful eruptions, usually accompanied by fever, arthralgias, and other systemic symptoms. Fewer than 50 cases have been reported in children, and has not been reported in association with pediatric lupus.
CASE REPORT: A 14 year-old African-American female presented with joint swelling and rash. Her knees had been painful and swollen for 2 weeks, and were stiff in the morning. On the day of admission, she noted swelling of many joints, along with a red rash on her hands, elbows, and feet. This rash was painless, and had occurred previously. On physical examination, the patient was afebrile with normal vital signs. There was a large, painless ulcer on the buccal mucosa. There were multiple firm, violaceous, well-circumscribed plaques on the elbows, hands, and feet. A painful small and large joint polyarthritis was noted. Abnormal laboratory studies included a WBC of 3,200/mm[sup3> (36% lymphocytes), ESR 77mm/hr, C3 26 mg/dL (90-187), C4 2.3 (16-45), ANA 1:1280, and multiple autoantibody titers (dsDNA, Smith, Smith/RNP, SSA, SSB, cardiolipin IgG, and [beta>2-Glycoprotein-1 IgG). A skin biopsy was revealed edema of the papillary dermis with early subepidermal bullae formation. There was a zone of acute inflammation beneath the edematous zone composed of neutrophils, with karyorrhexis, consistent with Sweet Syndrome.
COMMENT: Systemic Lupus Erythematosus was diagnosed based on physical and laboratory findings according to ACR criteria. The rash, however, was not typical of vasculitis, subacute cutaneous lupus, or discoid lupus. The patient subsequently developed episcleritis and nephritis, with recurrence of her rash during periods of active disease. Because of the ocular findings and anti-SSA and -SSB antibodies, secondary Sjogren Syndrome has been diagnosed. Sweet Syndrome should be considered a rare dermatologic manifestation of pediatric SLE and Sjogren Syndrome.