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Idiopathic Hypereosinophilic Syndrome With Cutaneous Manifestations: A Case Report.

Cureus

December 2024

Internal Medicine, Unidade Local De Saúde De São João, Porto, PRT.

Hypereosinophilic syndrome (HES) is marked by eosinophilic infiltration and the release of inflammatory mediators that cause damage to multiple organs. Despite careful evaluation of hypereosinophilia, the etiology of most cases remains undefined. Eosinophils may cause damage in almost all organs, and most patients present with dermatological manifestations.

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Eosinophilic myocarditis (EM), irrespective of its unique etiology, is marked by varying extents of eosinophil infiltration, frequently accompanied by peripheral eosinophilia. In some instances, the etiology remains undetermined, thus classified as idiopathic hypereosinophilic syndrome. The clinical manifestations are highly variable, ranging from mild or asymptomatic presentations to acute fulminant myocarditis or chronic restrictive cardiomyopathy.

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Wells' syndrome is a rare inflammatory disease characterized by recurrent, erythematous plaques with histological flame figures, which can be associated with idiopathic hypereosinophilic syndrome (IHES). We present a case of a nine-year-old boy who presented with a one-year history of an itchy rash on his legs associated with peripheral eosinophilia. The rash initially started as an annular plaque and developed raised borders with central hyperpigmentation.

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Introduction: Benralizumab is an interleukin 5-receptor-blocking drug registered for the treatment of eosinophilic asthma. It has proven efficient and safe in a small phase-II trial in hypereosinophilic syndrome and is currently being investigated in a larger, randomized phase-III trial. We report on real-world experience with benralizumab in 15 patients with severe Hypereosinophilic syndrome (HES) that were refractory to other treatments or on unacceptable steroid doses.

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