Systemic lupus erythematosus with erythema multiforme-like lesions and histiocytic necrotizing lymphadenitis--a case...

A 9-year-old Chinese girl with systemic lupus erythematosus presented initially with persistent cervical lymphadenopathy without any constitutional symptoms. The histology of the involved lymph node showed histiocytic necrotizing lymphadenitis and the initial clinical diagnosis of Kikuchi-Fujimoto disease was made. The patient subsequently developed fever, oral ulcerations, epistaxis, generalised myalgia and erythema multiforme-like skin lesions over the face and upper arms. Investigations showed anaemia, leucopenia, hypocomplementemia, a positive antinuclear antibody and a positive anti-double-stranded DNA antibody test. The clinical diagnosis was revised to systemic lupus erythematosus. The histological picture of histiocytic necrotizing lymphadenitis can occur in systemic lupus erythematosus and may be indistinguishable from that seen in Kikuchi-Fujimoto disease. The diagnosis of Kikuchi-Fujimoto disease should be confined to a benign clinical subset of histiocytic necrotizing lymphadenitis which resolves spontaneously without treatment. The occurrence of erythema multiforme-like lesions in lupus erythematosus is unusual and together with the characteristic serologic abnormalities, fits Rowell's syndrome.