Angiolymphoid Hyperplasia With Eosinophilia Vs Kimura Disease: Continuum of the Same Disease or a Distinct Entity-A Case Report With Literature Review.

Angiolymphoid hyperplasia with eosinophilia (ALHE) and Kimura disease were previously considered the same entities and are now considered a distinct disorder clinically and histologically. ALHE is a benign vasoproliferative disorder with unclear etiology. The clinical presentation of ALHE includes the involvement of skin and vascular structures sparing lymph nodes. It predominantly involves the head and neck region, extremities, and rarely orbit, oral mucosa, bones, and colon. On the other hand, Kimura disease is a rare benign chronic inflammatory disorder of unknown etiology that predominantly involves subcutaneous lymphoid masses and regional lymph nodes of the head and neck region. Both disorders are classified under hypereosinophilia (HE); however, Kimura disease is more associated with peripheral eosinophilia. It is tough to differentiate both the disorders clinically from each other and also from other HE syndromes including eosinophilic granulomatosis with polyangiitis and systemic HE syndromes. However, tissue diagnosis is the key to differentiation. Here, we describe a female at her 50s without any prior comorbidities, presented to our OPD with atypical multiple symmetrical soft tissue swellings which were of diagnostic dilemmas. She showed features of both ALHE and Kimura disease in investigations. As there is no specific recommendation for treatment, she was started with oral glucocorticoid and weekly methotrexate showing a good response in follow-up visit.