Journal Article

Kimura’s disease: case report of an Italian young male and response to oral cyclosporine A in an 8 years follow-up

Massi, Daniela, Emmi, Lorenzo, Liotta, Francesco, Maggi, Enrico, Benedetta Silvestri, Elena, Emmi, Giacomo, Chiodi, Michela, Di Paolo, Camilla, Beccastrini, Enrico

2013

Overview

Kimura’s disease is a benign chronic inflammatory disease, common in Asian males and rare in Western people. Clinically, Kimura’s disease is characterized by subcutaneous nodular lesions, usually localised in head and neck, often associated with regional lymphadenopathy. Peripheral blood eosinophilia and elevated serum IgE are often observed. We report a case of a 40-year-old Italian patient presenting with nodular subcutaneous lesions and peripheral eosinophilia. Based on clinical, histopathological and laboratory findings, a diagnosis of Kimura’s disease was made. The patient was treated with very low doses of cyclosporine A with no evidence of disease recurrence over the following 8 years. However, the discontinuation of cyclosporine A determined a relapse of the disease. The relevance of this case is due to the rarity of the disease in Italy, to its peculiar clinical presentation and, moreover, it is the first case in literature that has a good response to treatment with low doses of cyclosporine A, documented in an 8-year follow-up.

• Publisher: Springer-Verlag
• Subject: Administration, Oral / Adult / Angiolymphoid Hyperplasia with Eosinophilia - diagnosis / Angiolymphoid Hyperplasia with Eosinophilia - drug therapy / Case Report / Cyclosporine - therapeutic use / Glucocorticoids - therapeutic use / Humans / Immunosuppressive Agents - therapeutic use / Lymph Nodes - pathology / Male / Medicine / Medicine & Public Health / Prednisone - therapeutic use / Recurrence / Rheumatology / Treatment Outcome / Withholding Treatment