A Case of Juvenile Onset Sjoegren's Syndrome

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  • 小児Sjoegren症候群と考えた1例

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Abstract

We report a 9-year-old Japanese girl with Sjögren's syndrome. She had recurrent spiking fever and rheumatoid factor in her serum at the age of 7. One year later, she had recurrent annular erythemas on her feet without arthralgia. In a pediatric clinic, she had been treated with non-steroidal anti-inflammatory drugs under the diagnosis of juvenile rheumatoid arthritis. She had developed recurrent annular erythemas on her face and extremities beginning in October 1999, and she presented at our hospital in November 1999. Her serum was positive for anti-nuclear antibody, anti SS-A and SS-B antibodies and she had hypergammaglobulinemia. A biopsy specimen from an erythema on the left upper arm revealed liquefaction and degeneration of the basal layer, infiltration of lymphocytes and debris around the vessels and skin appendages in the dermis. At present, she manifests recurrent fever and annular erythemas. We believe that this patient has juvenile onset Sjögren's syndrome based on the recurrent fever, cervical swelling and annular erythemas, although subacute cutaneous lupus erythematosus is another differential diagnosis histopathologically.

Journal

  • Nishi Nihon Hifuka

    Nishi Nihon Hifuka 66 (4), 336-341, 2004

    Western Division of Japanese Dermatological Association

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