Painful indurated erythema suggestive of Kikuchi-Fujimoto disease in a patient with primary Sjögren's syndrome.
We report a patient with primary Sjögren's syndrome who developed pyrexia, cervical lymphadenopathy, and painful indurated erythema on the forehead, back, chest, abdomen, and limbs. Laboratory data showed an elevated erythrocyte sedimentation rate, C-reactive protein and CH50 in addition to existing autoantibodies including anti-nuclear antibody, anti SS-A antibody, and anti SS-B antibody. A skin biopsy specimen showed focal infiltration of histiocytes with non-neutrophilic karyorrhetic debris in the dermis and subcutaneous fat tissue. Immunohistochemically the infiltrated cells were stained for CD68, suggesting cutaneous involvement of Kikuchi-Fujimoto disease. All symptoms and laboratory data improved within three weeks after treatment with 20 mg/day of prednisolone. The present case suggests that a pathophysiological condition similar to Kikuchi-Fujimoto disease can develop during the long-term course of Sjögren's syndrome.
Miyashita
Yuko
Y
Department of Dermatology, Kawasaki Medical School, Kurashiki, Japan.
Yamaguchi
Masahide
M
Fujimoto
Wataru
W
D000328:Adult; D015703:Antigens, CD / Q000032:analysis; D015214:Antigens, Differentiation, Myelomonocytic / Q000032:analysis; D003937:Diagnosis, Differential; D004890:Erythema / Q000209:etiology; D005260:Female; D020042:Histiocytic Necrotizing Lymphadenitis / Q000209:etiology* / Q000276:immunology; D006801:Humans; D007150:Immunohistochemistry; D012859:Sjogren's Syndrome / Q000150:complications* / Q000276:immunology; D000097382:CD68 Molecule
D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; C067980:CD68 antigen, human; D000097382:CD68 Molecule