Annular Erythema Associated with Sjögren’s Syndrome Preceding Overlap Syndrome of Rheumatoid Arthritis and Polymyositis with Anti-PL-12 Autoantibodies

Abstract

A 44-year-old woman exhibited swelling with erythema on the bilateral upper eyelids. She was treated with prednisolone 20 mg/day at a different hospital. However, after the prednisolone was discontinued, the lesions relapsed. Four months after onset, the patient came to our clinic. A lip biopsy to investigate possible Sjogren’s syndrome (SS) confirmed lym phocytic infiltration of the minor salivary glands. Circulating autoantibodies to SS-A (anti-SS-A) were positive, but anti-SS-B antibodies were negative. She had no muscle weakness at any site of the body. Serum levels of creatinine kinase were not elevated. She was diagnosed with SS according to the Japanese Ministry of Health revised criteria for the diagnosis of SS (4). The lesions resolved with oral prednisolone 30 mg/day. When the prednisolone dose was reduced to 22.5 mg/day, several lesions of annular erythema (AE) appeared on the lower back (Fig. 1A). A biopsy of the AE lesion showed sleeve-like perivascular and periappendigeal lymphocytic infiltration, which was consistent with AE with SS (AESS) (Fig. 1B, C). The lesions resolved with oral prednisolone 30 mg/day and azathioprine 50 mg/day. Sixteen months after the onset of SS, arthralgia on the bilateral hip, knee, and ankle joints occurred. Oral prednisolone had been tapered to 10 mg/day, and oral azathioprine 50 mg/ day had been maintained. Physical examinations found swelling of the bilateral knee and ankle joints. Circulating anti-CCP antibodies were 453 U/ml (normal value < 15 U/ml). Serum levels of c-reactive protein and matrix metalloproteinase-3 Annular Erythema Associated with Sjogren’s Syndrome Preceding Overlap Syndrome of Rheumatoid Arthritis and Polymyositis with Anti-PL-12 Autoantibodies