Abstract:

Hypereosinophilic syndrome (HES) encompasses a group of rare diseases characterized by peripheral blood eosinophilia and eosinophil-mediated end organ dysfunction. HES-associated dermatoses, termed cutaneous HES (cHES), may play a role in diagnosis and management of HES. We sought to better define clinical and histopathological features of cHES by performing a retrospective review of 150 adult patients with HES evaluated at our institution from 2007 to 2018. Idiopathic HES was the most common subtype (133/150), followed by myeloproliferative (10), lymphoproliferative (5), and chronic eosinophilic leukemia (2). Sixty-one of 150 (41%) of patients had cHES. Demographics were: Caucasian (55/61); male (36/61); female (25/61); average age of diagnosis 49 (SD 18). Cutaneous dermatoses were noted in 41% (55/133) of patients with idiopathic HES, 80% (4/5) with lymphoproliferative and 20% (2/10) with myeloproliferative subtypes. Common clinical morphologies included urticaria (30%) and eczematous dermatitis (26%). At least one extracutaneous system was involved in 84% of patients with cHES: pulmonary (41%), ENT (26%), and nervous system (23%). Review of histopathology in the available 26 skin biopsies from 18 patients showed that the predominant pattern was spongiotic (31%) followed by superficial perivascular and interstitial dermatitis (27%). Inflammation was abundant in 50% of biopsies. Eosinophils were identified in 85% of biopsies, with 42% exhibiting abundant eosinophil infiltration. Flame figures were identified in 4/26 (15%). Two biopsies (8%) exhibited interstitial granulomatous dermatitis (both with abundant eosinophils); 2 (8%) showed eosinophilic fasciitis. Our study confirms a spectrum of clinical presentations and histopathology but no specific for cHES patterns. HES may be an underlying consideration in patients with eczematous or urticarial reactions, with or without abundant tissue eosinophils, in the setting of pronounced peripheral eosinophilia.

Financial Disclosure:
No current or relevant financial relationships exist.

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Jacqueline Zayas, MD, PhD