Table 1

Clinicopathological features of HES, Wells syndrome, CSS and EF HES
HES Wells syndrome CSS EF
Sex ratio (M:F) 9.1 1:1 1:1 2:1
Age HES can happen at any age, although it is more common in adults Wells syndrome usually affects adults CSS can present at any age, with the mean age of onset being 40 years EF usually affects adults between 20 and 60 years
Clinical features Skin rashes such as urticaria or angioedema Urticaria, cellulitis, annular plaques vesiculo-bullous lesions and edema Hypereosinophilia, asthma, pulmonary infiltrates, and clinical evidence of vasculitis Similar to scleroderma or systemic sclerosis
Histopathological features Eosinophilic infiltration with few lymphocytes, perivascular infiltration in dermis region, but not true necrotizing vasculitis Eosinophilic infiltrates and flame figures in the absence of vasculitis. perivascular eosinophilic infiltration in the dermis, but not true necrotizing vasculitis Peripheral blood eosinophils increase significantly, and neutrophil-rich leukocytoclastic vasculitis and granulomatous Numerous inflammatory infiltrations of lymphocytes and eosinophils within the fascia
Treatment Prednisone, hydroxyurea, chlorambucil and vincristine. Corticosteroids, calcineurin inhibitors, griseofulvin, H1 antihistamines, cyclosporine, dapsone Prednisolone, azathioprine and cyclophosphamide Prednisone
Clinical outcome More than 80% of HES patients survive five years or more It tends to resolve in weeks or months, usually without scarring. It occasionally recurs. In these recurrent cases, it can take years to ultimately resolve The mean five years mortality rate is 28% The prognosis is usually good in the case of an early treatment if there is no visceral involvement

Li et al.

Li et al. Diagnostic Pathology 2013 8:185   doi:10.1186/1746-1596-8-185

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