Kikuchi disease associated with Still disease

A 32-year-old white man with no previous medical past was admitted to our d epartment with an 8-month intermittent history of acute spiking fever with axillary, inguinal, and neck lymphadenopathy, arthritis, and cutaneous erup tion. Cutaneous examination showed nonpruriginous, indurated erythematous p apules affecting the face. ears. and trunk, suggestive of subacute lupus er ythematosus (Figs la and Ib). On general physical examination. no abnormali ties were observed except for arthritis of the right ankle and, a few month s later, arthritis of the left knee. Biological investigations revealed a r aised erythrocyte sedimentation rate with a serum ferritin level at 800 ng/ mL (normal < 200 ng/mL), a white blood cell count of 12,500/mm(3) (with 80% neutrophils, 16% lymphocytes, and 4% monocytes), a hematocrit of 40%, and a hemoglobin level of 13.1 g/dL. Electrolytes and liver function tests were normal. Antinuclear antibodies and antibodies to deoxyribonucleic acid (DN A) were negative. Rheumatoid factor was absent. No evidence of the followin g infections/organisms was found on serologic screening: syphilis, Streptoc occus, Toxoplasma, Mycoplasma, Brucella, Borrelia burgdorgferi, spotted fev er, leptospirosis, hepatitis B and C virus, parvovirus, human immunodeficie ncy virus (HIV) 1 and 2, human T-cell lymphotropic virus (HTLV) 1, Epstein- Barr virus (EBV), cytomegalovirus (CMV), herpes simplex virus (HSV) 6. Immu noglobulin M (IgM) HSV(1 + 2) by enzyme-linked immunosorbent assay (ELISA) analysis with 1 year follow-up was still detected. The articular synovial f luid of the involved ankle and knee was inflammatory but sterile. A bone ma rrow biopsy specimen revealed no lymphomatous involvement. Abdominal and ch est scans were normal. Skin biopsy of a facial nodule showed, in the dermis and in the subcutaneous fat tissue, a dense perivascular and periadnexal i nfiltrate composed of numerous plasmacytoid histiocytes and large- or mediu m-sized lymphocytes (Figs 2a and 2b). No epidermal changes, edema in the pa pillary dermis, or vasculitis were found. Immunofluorescence staining of fr ozen tissue sections of a facial nodule showed granular deposits of C3. Ing uinal lymph node biopsy revealed, in the paracortical area, focal necrosis with a proliferation of transformed lymphocytes, histiocytes, and numerous nuclear debris typical of histiocytic necrotizing lymphadenitis (Figs 3a an d 3b). On immunohistochemical staining examination, CD68 was positive in pa thologic paracortical areas. Few mononuclear cells were positive for CD3, C D8, and L-26 (Fig. 4), Polymerase chain reaction (PCR) amplification did no t show human herpes simplex viruses in the skin biopsy specimen, and cultur e was also negative. On account of the viral herpetic persistent serodiagnosis, a treatment by v alaciclovir then aciclovir at a dose of 800 mg/day was administered, but wa s not efficient. Clinical features improved rapidly with oral prednisolone therapy. 60 mg/day, but a relapse was observed a few weeks later during a d ecrease in steroid dose.