The mystery of recurrent Kimura’s disease: A rare case with histopathological insights

Kimura’s disease is a rare, chronic inflammatory disorder of unknown etiology, classically presenting as painless subcutaneous nodules in the head-and-neck region, often associated with lymphadenopathy, peripheral eosinophilia, and elevated serum immunoglobulin E levels. It predominantly affects young Asian males, making its occurrence in females uncommon and diagnostically challenging. We report a rare case of recurrent Kimura’s disease in a 34-year-old woman presenting with a progressively enlarging, painless left preauricular and parotid-region mass causing significant esthetic deformity. The patient had undergone multiple surgical excisions at other centers over 3 consecutive years, with rapid and florid recurrences and a histopathological diagnosis of foreign-body giant cell reaction. Imaging evaluation suggested a benign neural tumor, further complicating the diagnostic process. Definitive diagnosis was established following complete surgical excision and detailed histopathological examination, which revealed characteristic features including florid lymphoid follicular hyperplasia with prominent germinal centers, dense eosinophilic infiltrates with microabscess formation, vascular proliferation, and stromal sclerosis, confirming Kimura’s disease. The lesion was found adherent to the parotid fascia with extensions into the gland and encasement of facial nerve branches, necessitating meticulous dissection to preserve facial nerve integrity. Postoperatively, the patient developed a transient salivary fistula. This case highlights the diagnostic complexity, atypical demographic presentation, and recurrence-prone nature of Kimura’s disease. It underscores the critical role of histopathology in diagnosis and the need for long-term follow-up and consideration of adjuvant medical therapy to reduce recurrence risk.