HYPEREOSINOPHILIC SYNDROME

By Gary M. White, MD

Hypereosinophilic Syndrome in an elderly woman.

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Hypereosinophilic Syndrome (HES) is a systemic disorder featuring peripheral eosinophilia and eosinophilic infiltration of numerous organs, including the heart, lungs, skin, kidneys, liver, GI tract and nervous system.

Clinical

Cutaneous lesions occur in about half of patients. The most common findings are pruritic erythematous papules. Vesicobulla, diffuse eczema, nodules, erythroderma, EAC, pruritus, persistent mucosal erosions and vasculitis have all been reported.

Diagnosis

Criteria for diagnosis is

1. Peripheral eosinophilia 1.5 x 10 to the 9/L for more than 6 months,
2. No evidence of parasites, allergic diseases, or T-cell proliferations; and
3. Presumptive signs of organ involvement.

Treatment

Treatment is indicated for significant organ dysfunction. Symptomatic skin disease may also be an indication. Initial therapy is usually with prednisone, e.g. 1 mg/kg/day. Low dose or intermittent pulse therapy may be used. If needed, prednisone may be combined with hydroxyurea or other cytotoxic agents. Other treatments have included vincristine, etoposide, chlorambucil. interferon alpha and cyclosporine.

Additional Pictures