Sjöegren syndrome with edematous erythema

The patient was a 20-year-old woman presenting edematous erythemas on her left cheek with a high fever. The diagnosis was subclinical Sjögren syndrome based on high titers of anti-nuclear antibody (× 1280: speckled type), anti-SS-A, and anti-SS-B antibodies, and on positive Schirmer test, sialography and lip biopsy. Thirty-nine patients (including our case) with this syndrome accompanied by the erythema had been previously reported in Japan. The clinical and histopathological findings of these patients were discussed. There had been 13 reported patients with annular erythema and 11 patients with edematous erythema. However, in some patients with annular erythema, eruptions were not similar to Darier's annular erythema and “coat-sleeve”-like lymphocytic infiltration was absent histologically. Therefore, such erythema should be carefully observed and described.