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Hypereosinophilic syndrome (HES) is a group of rare blood disorders characterized by persistent peripheral blood eosinophilia (eosinophil count of 1.5 × 109/L or greater) and evidence of end-organ involvement. HES is a diagnosis of exclusion, and clonal eosinophilia and reactive eosinophilia must first be ruled out. Confirmation of HES relies on assessing blood and bone marrow morphology, cytogenetics, flow cytometry, and T-cell clonality. HES can present with primarily cutaneous findings.1  Lymphocyte-variant HES (L-HES) is a distinct subtype, characterized by aberrant clonal T-cell populations that produce eosinophil-promoting cytokines.2 Currently, the first-line treatment for L-HES is systemic steroids.3 However, a recent study showed that L-HES is more steroid resistant than other forms of HES, with an odds ratio of steroid response of only 0.34 compared with idiopathic HES.4  At present, there is no standard therapy for steroid-refractory L-HES. Mepolizumab, an anti–interleukin (IL) 5 monoclonal antibody, is the only agent shown to be an effective steroid-sparing agent in a randomized clinical trial of patients with HES.5 Interferon α, cyclosporine, imatinib, methotrexate, alemtuzumab, hydroxyurea, cyclophosphamide, fludarabine, and Janus kinase (JAK) inhibition have all been reported in small numbers of patients, with varying degrees of efficacy.3, 6 In contrast, patients with myeloid HES, in which malignant myeloid clones express platelet-derived growth factor, often respond to imatinib therapy.1  We present a case of L-HES showing skin presentation, chronicity of clinical course, and successful treatment with mycophenolic acid (MFA). To our knowledge, this represents the first case in which cutaneous manifestations and eosinophilia have been successfully managed with MFA.

Lymphocyte-variant hypereosinophilic syndrome presenting as chronic dermatitis and responding to mycophenolic acid