Table 1 Clinicopathological features of HES, Wells syndrome, CSS and EF HES
From: Recurrent cutaneous necrotizing eosinophilic vasculitis: a case report and review of the literature
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 |