Idiopathic hypereosinophilic syndrome with cutaneous involvement: a comparative review of 32 cases

Abstract

Although idiopathic hypereosinophilic syndrome (HES) is uncommon, we studied the clinical characteristics of this disorder in patients with cutaneous involvement. We chronicle the case of a patient with diffuse skin rash due to idiopathic HES from our clinical experience. Furthermore, a systematic literature search of the medical databases PubMed and Google Scholar was conducted. A total of 32 cases fulfilled the inclusion criteria. The data on patients’ characteristics, epidemiology, clinical features, diagnosis, treatment and outcome were collected and analysed. This review illustrates that physicians should maintain a high index of clinical suspicion for idiopathic HES in patients presenting with dermatological lesions and hypereosinophilia, without an obvious cause. Randomised clinical trials are warranted to outline a generalised and efficient therapeutic approach in these patients. Additionally, this paper highlights the need for population-based studies to delineate the magnitude and scope of this association.

Urticaria: A Narrative Overview of Differential Diagnosis

“…Hypereosinophilic syndrome (HES) is an uncommon, multisystemic and heterogenous group of disorders, first described by Hardy and Anderson in 1968 [ 74 ]. HES can divided into many subtypes (e.g., eosinophilic granulomatosis with polyangiitis overlap, myeloid variant, lymphocytic variant, single-organ, idiopathic) [ 75 ]. Idiopathic HES is defined by the presence of: (i) long-lasting (>6 months) blood eosinophilia (>1.5 × 1053/L); (ii) organ damage and/or dysfunction that can be linked to eosinophilia and iii) lack of alternative root [ 76 ].…”

An Approach to Hypereosinophilic Syndrome Presenting With Cutaneous Features

“…In a series of 32 cases of I-HES, presentations on the skin were reminiscent of those reported in the L-HES variant, resembling urticarial or eczematous lesions. 39 Numerous published cases of I-HES report cutaneous vascular symptoms, including Raynaud's phenomenon, livedo reticularis, purpura, ulceration, and necrosis. [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.…”

“…In a review of 32 cases of I-HES presenting with cutaneous symptoms, most had clinical signs or symptoms suggestive of organ involvement, or further investigation revealed organ disease. 39…”

Clinical and histopathological features of hypereosinophilic syndrome with cutaneous involvement: The Mayo Clinic Experience

“…The differential diagnosis for eosinophilia with the aforementioned cutaneous findings ranges from common dermatoses such as atopic, allergic, or irritant contact dermatitis and acute or chronic urticaria to Wells syndrome, Hyper IgE syndrome, or other dermatoses associated with immune dysfunction such as CARD9 deficiency. The histopathology of cHES has been described mostly in small case series 11,12 totaling 15 patients and single case reports 6 …”