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Box 2 Main clinical manifestations of HES

From: French guidelines for the etiological workup of eosinophilia and the management of hypereosinophilic syndromes

Cutaneous: pruritus, eczema, urticaria, angioedema, bullae, ulceration of the limbs or mucous membranes, splinter hemorrhages, fasciitis, livedo, purpura.

Pulmonary: asthma, bronchiolitis, bronchitis, bronchiectasis, interstitial lung disease.

Cardiac: myocarditis, pericarditis, valvular disease, endomyocardial fibrosis, dilated cardiomyopathy, intracavitary thrombus, coronary artery vasospasm.

Neurological: ischemic cerebrovascular disease (usually bilateral and of watershed distribution), peripheral neuropathy, myelitis.

Digestive: eosinophilic esophagitis, eosinophilic gastritis, eosinophilic enteritis and eosinophilic colitis, eosinophilic ascites, eosinophilic cholangitis.

Arterial and/or venous thrombosis.

Rheumatologic: arthritis, tenosynovitis, myositis.

Thromboangiitis obliterans–like vasculopathy or eosinophilic vasculitis (excluding EGPA or PAN).

  1. Hypereosinophilic asthma with sinonasal polyposis are common manifestations of T2 diseases, usually restricted to airways in most patients but can also be part of HES.
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172