kimura’s disease

Angiolymphoid hyperplasia with eosinophilia (ALHE) is characterized by irregularly-shaped blood vessels with an inflammatory infiltrate. While absent from normal skin, plasmacytoid dendritic cells (pDCs) infiltrate the skin upon injury and during several infectious, inflammatory, and neoplastic entities. In addition to providing anti-viral resistance, pDCs link the innate and adaptive immune responses. In Kimura’s disease (KD), pDCs have been reported to occur. Here, we investigate pDCs in ALHE. Five ALHE and comparable 4 KD cases were immunohistochemically tested for pDC occurrence and type I IFN production using anti-BDCA-2 and anti- myxovirus protein A (MxA) antibodies, respectively. A semiquantitative scoring system was used. Plasmacytoid dendritic cells were present in all ALHE and KD cases with no statistically significant differences, while MxA expression was weak and patchy in most ALHE and KD cases. pDCs are recruited into the lesions of ALHE. Despite the diminished ability to produce type I IFNs, the consistent presence of pDCs in all ALHE cases is in favor of some role of these cells in ALHE pathogenesis.

Herein we report a case of Kimura’s disease with unusual manifestations. A 46-year- old Chinese man presented with mass in scrotum which gradually increased in size for approximately 7 years. A computerized tomographic scan of abdomen revealed a soft-tissue density shadow in scrotum and enlargement of lymph nodes in groin and retroperitoneum. The scrotal mass was excised and the biopsy specimen revealed angiolymphoid hyperplasia with infiltration of eosinophils. In conclusion, clinical doctors should pay attention that the patient with eosinophilia and scrotal mass could be indicative for Kimura’s disease.

Kimura’s disease (KD) is an allergic inflammatory disorder of unknown etiology endemic in Orientals. Kimura’s disease was first mentioned by Kimm and Szeto in China in 1937. Kimura’s disease is commonly encountered in Asia and is mostly reported in Japan, China, Singapore and Honkong. However, only a few cases have been reported in the Indian subcontinent.
Case Report: A case of Kimura’s disease in a young male managed by surgery is reported in addition to a literature review.
Diagnosis is made on the basis of histopathological analysis, clinical presentation, and laboratory investigations. Long term follow-up is required as Kimura’s disease is prone for recurrence.

Kimura's disease (KD) is a rare idiopathic condition of unknown etiology. The disease is characterized by swelling and lesions in the head and neck region, with involvement of the subcutaneous soft tissue, major salivary glands and lymph nodes. Patients almost always have eosinophilia and elevated serum immunoglobulin E levels. The diagnosis is established by biopsy. KD is usually self limiting. We report a case of Kimura’sdisease in a female and review the literature. Thepresent report highlights the need for increased awareness byall otolaryngologist of this clinically impressive entity.