[A case of Kimura disease treated with interferon and general corticoid therapy]

A case of Kimura's disease that occurred in a 5-year-old Caucasian boy after a tick bite is reported. When the child was 16 years old, symptoms developed. They included voluminous bilateral neck and head lymph nodes associated with hypereosinophilia (1,640/mm3), and increased IgE levels (18,866 KU/L). Clinical and immunological effects of treatment by interferon-alpha and steroids are presented.

Pathological and histological examination showed typical features of dense lymphoid cell infiltrates containing many eosinophils, mast cells, and vascular hyperplasia. Percentages of CD4+, CD27-, CD7- cells were increased In the blood and lymph nodes, showing a profile typical of TH2. IL-5 production by these cells was markedly increased and was inhibited by IFN-alpha and IFN-beta in vitro. No etiology was found. The role of antigens of Ixodes ricinus is discussed. Three surgical excisions of adenopathies were not successful. Treatment by IFN-alpha (Introna, Schering-Plough, 5.10(6) U/week) and a bolus of methylprednisolone hemisuccinate (1 g/month) was started. Eight months later, the size of the lymph nodes had decreased; however, eosinophil counts and ECP and IgE levels were still high. The decrease in corticosteroid induced a subsequent, slight increase in the size of the lymph nodes and a marked increase in ECP.

This is the first description of treatment of Kimura's disease by interferon-alpha and steroids. The disease outcome does not suggest that interferon-alpha may predominate.