Mushtaq A Badami, Khalid Mahmood, Uzma Ghori, Ashfaq Ahmed.
Kimura`s Disease with Atypical Presentation.
J Coll Physicians Surg Pak Jan ;11(9):575-7.

Kimura`s disease is a benign, slow growing vascular tumor with marked predilection for the head and neck. It has uncertain etiology, manifested clinically by one or more inflammatory nodules, lymphadenopathy, peripheral eosinophilia and elevated IgE levels. A typical case of this rare disease, with some unusual findings is presented. Although a rare disease, a good number of cases have been reported especially among Asian population.

A 65-year old male was presented in outpatient clinic with generalized pruritis and bilateral inguinal swellings for four months. He also developed progressive bilateral pedal edema which was non-pitting type for three months. He had low-grade fever at night associated with sweating for the past three months. On inquiring he stated that he had decreased appetite and losing weight for the past one month. Few days after his admission he developed swelling in his right axilla and right parotid gland which subsided spontaneously. But after few days he again developed swelling of left parotid gland.

His physical examination revealed pulse of 104/min, blood pressure 110/70 mmHg and temperature 100oF. Right axillary lymph node was palpable about 2.0 x 3.0 cm in size, hot, but non-tender, soft in consistency. He also had bilateral enlarged inguinal lymph nodes, 2.0 x 3.0 cm on the right and 2.0x1.0 cm on the left side. Swellings were non tender, mobile, soft in cosistency, overlying skin temperature and texture was normal.

His parotid glands were also enlarged bilaterally, 2.0 x 2.5 cm on the right and 2.0 x 2.0 cm on the left side. These were hot, but non-tender, soft in consistency, and overlying skin was normal in texture. Skin examination revealed small subcutaneous papules all over body. Abdominal examination revealed multiple lumps on deep palpation. Respiratory, cardiovascular and CNS examinations were normal.

Investigations showed Hb 10gm%, TLC 11900/mm3 with moderate eosinophilia of 12% and ESR was 40 mm/1st hr. Other investigations and x-ray chest were normal. Ultrasound of abdomen showed paraaortic, parapancreatic and bilateral inguinal lymph node enlargement. Liver and spleen were normal. CT scan of abdomen also revealed extensive paraaortic and retroperitoneal lymphadenopathy from celiac level to aortic bifurcation, also in both inguinal and internal iliac regions. Bone marrow aspirate showed adequate cellularity, no evidence of lymphocytic infiltration. Blasts were 4%. Biopsy was taken from inguinal lymph node, which revealed focal hyperplasia of lymphoid follicles with few germinal centres, that were well vascularized and contained polymorphs as well as eosinophils with no evidence of malignancy. There were benign reactive changes with features suggesting angiolymphoid hyperplasia with eosinophilia (Kimura`s disease). Axillary lymph node biopsy was also sent for confirmation of diagnosis and to rule out the possibility of lymphoproliferative disorders. That biopsy also showed similar findings, confirming the diagnosis of Kimura`s disease.

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