Clinical Images: Giant mass on the forehead in Kimura disease.

Abstract

The patient, a 68-year-old Asian man with a history of nephrotic syndrome, presented to our department with multiple subcutaneous masses that developed over the past 50 years. The masses were painless, soft, and predominantly located in the head. The mass on the forehead was dome-shaped and had a diameter of 7 cm and a height of 3 cm (A). Blood tests showed eosinophilia and elevated immunoglobulin E (IgE) levels. Serum immunoglobulin G4 levels were normal. Ultrasonography showed heterogeneous masses with marked associated internal hyperemia (B). Magnetic resonance imaging showed T1-weighted isointense and T2-weighted hyperintense masses. A needle biopsy of the forehead mass showed stromal fibrosis, multiple lymphoid follicles, and infiltration of inflammatory cells, mainly eosinophils and lymphocytes. The patient was diagnosed with Kimura disease (KD). The masses shrank rapidly after administration of 30 mg/day of prednisolone for 2 weeks. KD is a rare chronic inflammatory disorder mainly affecting young Asian men. It presents as subcutaneous masses, typically in the head and neck, and is associated with eosinophilia and elevated IgE levels (1). It often affects the cervical lymph nodes and salivary glands (1). It is often associated with renal involvement, such as nephrotic syndrome (2). Glucocorticoids, surgical excision, and radiation therapy are the treatments of choice (3). The diagnosis of KD can be difficult because of its rarity. KD should be considered as a differential diagnosis when patients present with painless subcutaneous masses in the head and neck region with eosinophilia and elevated serum IgE levels.