The peripheral neuropathy associated with HES has been documented with increased frequency but little is known about its natural history. Neurolo¬gical symptoms may be the primary manifestations in HES. A variety of neuropathy has been de¬scribed, including multiple mononeuropathy, distal symmetrical motor neuropathy, and radiculopathy in the peripheral nervous system. Most subjects have had very mild peripheral neuropathy either by clinical or EMG criteria. Nerve biopsy or autopsy has shown histopathological findings consistent with wallerian degeneration, axonal degeneration, de- myelination, and vasculitis. However, the patho¬genic mechanisms of eosinophil in peripheral neuro¬pathy are still unclear. Eosinophils contain cytotoxic granules that release eosinophil cationic protein, which is partially responsible for thromboembolism, neurotoxic protein, and major basic protein.
It has been suggested that the neuropathy is progressive during the period of hypereosinophilia, but may show some clinical resolution after corticosteroid treatment. The neurological symptoms, especially, neuropathies, take the longest to recover. The rare form of very severe neuropathy shows poor recovery.
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Cutaneous involvement occurs in more than 50% of patients with HES. The most common lesions are erythematous pruritic papules and nodules, and angioedematous and urticarial lesions; the latter are associated with a better prognosis. Other types of skin lesions are blistering lesions and vasculitic lesions that result from dermal microthrombi. Mu- cosal ulcerations, when they occur in HES, can cause significant morbidity and are difficult to treat. Skin biopsy specimens usually show eosinophil-rich mixed cellular infiltrate.
Table 1. The differential diagnosis of cutaneous disease with eosinophilia
Diseases with peripheral and/or tissue eosinophilia
Atopic diseases Parasitic diseases
Eosinophilia myalgia syndrome
Toxic oil syndrome
Urticaria and angioedema Mastocytosis
Cutaneous T cell lymphoma
Reactions to arthropod bites and stings
Diseases histologically characterized by tissue eosinophilia
Annals of Dermatology Vol. 20, No. 3, September 2008
Kimura’s disease and angiolymphoid hyperplasia with eosinophilia
Wells’ syndrome (eosinophilic cellulitis)
Eosinophilic pustulosis (Ofugi’s disease and erythema toxicum neonatorum)
Eosinophilic ulcer of the tongue
Table 1 summarizes the differential diagnoses of cutaneous disease with eosinophilia. Sometimes HES shows clinical and electrophysiological features mimicking systemic vasculitis. The systemic necro- tizing vasculitis such as Churg-Strauss syndrome, periarteritis nodosa was excluded because none of the following manifestations were documented in our patient: fever, constitutional symptom, clear cut pulmonary affectations or asthma, sinusitis or rhinitis, and little response with corticosteroids. Also biopsy showed no vascular change in our case, and systemic necrotizing vasculitis was ruled out.
Glucocorticoids have been used as initial and maintenance therapy. For patients unresponsive to predinisone, hydroxyurea is added. Vincristine, INF- a, imatinib mesylate (Gleevec), and a tyrosine kinase inhibitor were found to be effective for certain patients with HES. The aim is to maintain the leukocyte count at less than 10*109/L, with a normal eosinophil count. Cardiac damage is the most important factor in the prognosis of HES. Improvement in peripheral eosinophilia correlates with an improved cardiac status. levitra professional
Recently two pathogenic variants of HES have been defined: myeloproliferative HES and lympho- proliferative HES. Hematologic malignancy has been diagnosed in patients, 9~12 years after they were diagnosed with HES. Though we could not perform a bone marrow evaluation to rule out hematologic malignancy, there was pure normal eosinophil infiltration in the skin biopsy specimens and no atypical eosinophil or eosinophilic precursor cell in the peripheral blood smear. From these findings, we could make a differential diagnosis of eosinophilic leukemia. With careful follow-up tests and a low-dose maintenance steroid therapy, the prognosis for our patients appears good.