A Unique Case of Idiopathic Hypereosinophilic Syndrome in a Patient Presenting with Chronic Urticaria
Sunday, March 6, 2016
South Exhibit Hall H (Convention Center)
Stephanie N. Hudey, M.S., Hana B. Niebur, MD, Jennifer W. Leiding, MD
Rationale: Hypereosinophilic syndromes (HES) comprise a spectrum of disorders characterized by prolonged eosinophilia with evidence of end-organ damage. Dermatologic manifestations are among the most common presenting symptoms. We describe a patient who after extensive evaluation was found to have idiopathic HES.

Methods: Retrospective chart review was performed to include clinical and laboratory data. 

Results: The patient is a 59 year old female with a 2 year history of daily hives with progressive erythroderma, alopecia, and bilateral hand tremor. Initial evaluation revealed IgE 28,933 kU/L, AEC 1600 cells/mm3, and she was treated for chronic urticaria with high-dose antihistamines without response. Transition to methotrexate and prednisone improved IgE level but she continued to have symptoms. Scleral icterus, hepatomegaly with 22cm liver span on CT scan, and elevated liver transaminases soon developed. Full-thickness skin biopsy demonstrated subacute spongiotic dermatitis with numerous eosinophils.  Serologic examination showed absence of anti–nuclear, -myeloperoxidase, -proteinase 3 antibodies and normal vitamin B12, tryptase and troponin levels. Bone marrow biopsy revealed intact trilineage hematopoiesis and no mutations in PDGRFB, FGFR1, c-kit, JAK2 V617F, PDGFRA, and FIP1L1were identified. Eosinophil and IgE levels and dermatologic abnormalities improved with high-dose steroids but liver dysfunction progressed. She was transitioned to hydroxyurea and liver biopsy was recommended. 

Conclusions: HES is an uncommon disease that must be considered in patients with chronic urticaria, especially in those unresponsive to antihistamines, with prolonged eosinophilia or other organ dysfunction.