Hypereosinophilic syndrome complicated by severe vascular damage and gangrene

Abstract

Hypereosinophilic syndrome (HES) is a complex multisystem disease characterized by sustained overproduction of eosinophils. A 40-year-old woman presented with digital ischemia and gangrene on her distal fingers and toes. We diagnosed HES on the basis of marked eosinophilia, accumulation of eosinophils in organs, and cutaneous eosinophilic vasculitis after having excluded all differential diagnoses. On digital subtraction angiography, occlusion of several arteries of both lower legs was noted. HES may be associated with severe vascular damage including gangrene. The occurrence of digital gangrene is a differential diagnostic challenge that should also include investigations of blood parameters, such as eosinophils.

An Approach to Hypereosinophilic Syndrome Presenting With Cutaneous Features

“…Numerous published cases of I-HES report cutaneous vascular symptoms, including Raynaud’s phenomenon, livedo reticularis, purpura, ulceration, and necrosis 47–52 . Histopathology in several cases has confirmed the presence of eosinophilic vasculitis, proposing that it may be a significant feature of HES 48,53,54 . Eosinophilic infiltration of the hair follicle contributing to a diffuse-type hair loss has been reported as the presenting feature in 1 case of I-HES 55 .…”

“…[47][48][49][50][51][52] Histopathology in several cases has confirmed the presence of eosinophilic vasculitis, proposing that it may be a significant feature of HES. 48,53,54 Eosinophilic infiltration of the hair follicle contributing to a diffuse-type hair loss has been reported as the presenting feature in 1 case of I-HES. 55 The presentations reported in cases limited to skin involvement seem to reflect the wide range seen in L-HES and I-HES.…”

An Update on Eosinophilic Esophagitis: Etiological Factors, Coexisting Diseases, and Complications

“…Certain conditions such as inflammatory mediators, genetic susceptibility, environmental factors, atopic conditions, dietary products, esophageal microbiome, and oral immunotherapy have been associated with a higher risk of EoE formation. However, various other conditions with distinct clinical and histological characteristics have also been identified to be associated with esophageal eosinophilia such as eosinophilic gastrointestinal diseases, proton pump inhibitor (PPI)-responsive esophageal eosinophilia, drug hypersensitivity, pemphigus, connective tissue disorders, graft-versus-host disease [41], and hypereosinophilic syndrome, a rare group of conditions that are associated with persistent high eosinophilia with eosinophil counts >1,500/μL, which most commonly affects the heart, central nervous system, skin, and respiratory tract [42].…”