Clinical features of Sjögren’s syndrome associated with recurrent annular erythema

Abstract

Our objective was to describe the clinical features of Sjögren’s syndrome (SS) with recurrent annular erythema which resembles subacute cutaneous lupus erythematosus (SCLE), and determine a possible association of anti-SS-A/Ro and/or SS-B/La antibody titers with the episodes of cutaneous manifestation. Recurrent annular erythema was observed in 4% (six patients; five females and one male) of our 143 patients diagnosed as primary SS. All the patients developed annular erythema on the facial area as their initial manifestation when they were between 21 and 31 years old. They had few subjective sicca symptoms, but both anti-SS-A/Ro and SS-B/La antibodies were positive and parotid sialography showed typical findings for SS (subclinical SS). Parotid gland swellings had developed in five of the patients during their follow-up periods, (3–12 years). In addition to the facial area, most patients repeated the cutaneous episodes on extremities and palmar, plantar or auricular areas. Skin biopsy was performed in three patients and the common findings were mononuclear cell infiltrations in the dermis with few epidermal changes. Transient leukopenia (four patients), low titers of anti-DNA antibodies (one patient) and chronic false-positive results of serological tests for syphilis (one patient) were observed. Two of our patients, therefore, temporarily fulfilled four items of the ARA classification criteria for systemic lupus erythematosus (SLE), if their facial erythema was considered as malar rash. Serum antibodies to 52 kDa SS-A/Ro peptides (80%) were more frequently detected in the five patients examined by enzyme-linked immunosorbent assays (ELISA), compared with those to 60 kDa SS-A/Ro peptides (40%). Furthermore, we could serially determine serum anti-SS-A/Ro and SS-B/La antibody titers in three patients by using ELISA for 3 or 4 years. Annular erythema usually developed when the antibody titers became relatively high and disappeared after the treatment with oral prednisolone which suppressed the antibody titers. We could observe five pregnancies in our three patients and all the patients developed annular erythema during their pregnant periods. Their five infants, however, were free from any complications such as neonatal lupus erythematosus and congenital heart block. We conclude that annular erythema is a rare manifestation of SS and develops in relatively young patients who are subclinical for sicca symptoms. The cutaneous episodes seemed to relate with anti-SS-A/Ro and/or SS-B/La antibody titers. Some of the erythema observed in SS patients may belong to SCLE, but they do not usually develop typical SLE. The possibility that SS may be more frequent in the SCLE patients remains to be determined.