Successful use of mepolizumab for severe hypereosinophilic vasculitis with c-ANCA positivity in a previously healthy 7-year-old boy.

An unusual case of a pediatric patient with severe eosinophilic vasculitis causing digital ischemia is reported. The patient responded well to the anti-IL-5 agent mepolizumab, lending support for use of mepolizumab in pediatric patients with hypereosinophilic syndromes.

Hypereosinophilic vasculitis with Raynaud phenomenon presentation: a case report.

BACKGROUND: Previous case series have reported idiopathic eosinophilic vasculitis as a potential manifestation of hypereosinophilic syndrome (HES). This condition is characterized by digital necrotizing, systemic vasculitis that affects varying-sized blood vessels. This report presents our experience in treating a patient with eosinophilic vasculitis. CASE PRESENTATION: We describe the case of a 23-year-old man who presented with idiopathic HES, which manifested as digital ulcers and peripheral ischemia in both the upper and lower limbs, without the involvement of other organ systems. After ruling out primary and secondary causes of eosinophilia, a diagnosis of HES was established. Our patient has shown a positive response to corticosteroid therapy. CONCLUSIONS: Our case contributes to the existing evidence about diagnosing idiopathic eosinophilic vasculitis in patients with HES. We observed a favorable response to corticosteroid treatment in our patient.

Recurrent cutaneous eosinophilic vasculitis characterized by annular purpuric lesions: A case report.

A 71-year-old woman presented with a persistent, intensely pruritic cutaneous eruption localized on the palmoplantar regions, lips and palate. The histological findings allowed to make the diagnosis of recurrent cutaneous eosinophilic vasculitis, a very rare cutaneous vasculitis characterized clinically by multiple erythematous or purpuric erythematous papules or plaques or angioedema with a relapsing course in the absence of systemic involvement and histologically by a necrotizing vasculitis of the dermal small vessels with a dominant eosinophilic infiltration. The patient was treated with oral methylprednisolone and pentoxifylline which led to a rapid resolution of the cutaneous lesions.

Eosinophilic granulomatosis with polyangiitis: A review.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, multi-system, inflammatory disease, belonging to the group of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). Previously known as Churg-Strauss syndrome, EGPA is characterised by late-onset asthma, eosinophilia and vasculitis affecting small-to-medium vessels. This disease behaves differently in many aspects to the other AAV and is often excluded from AAV studies. The disease is poorly understood and, due to it rarity and unique manifestations, there has been limited research progress to optimise our understanding of its complex pathogenesis and ability to develop management options - although the success of interleukin-5 inhibitors such as Mepolizumab has been a welcome development. The pathophysiology also appears to be different to other forms of AAV and hence management strategies that work for AAV may not fully apply to this condition. There is no current standard therapy for EGPA although corticosteroids are almost universally used for treatment alongside other agents and encouraging modes of treatment continue to evolve beyond glucocorticoid immunosuppression (including interleukin-5 inhibition). There is therefore a significant ongoing unmet need for efficacious steroid-sparing immunosuppressing agents. The prognosis also diverges from other forms of AAV, and we discuss the pathophysiology, clinical features and diagnosis, management and prognosis in this article.

Recurrent cutaneous necrotising eosinophilic vasculitis.

Recurrent cutaneous necrotising eosinophilic vasculitis (RCNEV) is a rare disease that was first described in 1994. We report a case of RCNEV treated with corticosteroid, and 18 cases that we identified in the literature. Our review of the literature shows that RCNEV was frequently identified in middle-aged females from Asia and usually presents as erythematous to purpuric papuloplaques, angio-oedema on the extremities, as well as peripheral eosinophilia. Histopathologically, RCNEV is characterised by exclusively eosinophilic infiltration around the vascular plexus, the absence of leukocytoclasis and fibrinoid degeneration of vascular walls. Although, RCNEV responds to corticosteroid treatment, relapses have occurred during dose tapering. We also discuss the mechanisms of vascular destruction, the differential diagnosis and steroid-sparing therapies for RCNEV.

An uncommon form of localized colonic eosinophilic vasculitis with extensive thrombosis of the spleen and liver: A case report and literature review.

INTRODUCTION: Localized vasculitis of the gastrointestinal tract is an uncommon disease that mainly presents as polyarteritis nodosa and is mainly located on small bowel and gall bladder. Localized eosinophilic vasculitis of the colon, which needs surgical intervention, has never been reported before. CASE PRESENTATION: A 40-year-old man was diagnosed with localized eosinophilic vasculitis of the colon with an initial presentation of necrotizing colitis of ascending colon. After right hemicolectomy, extensive thrombosis of the liver and spleen occurred with the presentation of abdominal pain. The histopathological analysis revealed ischemic colitis with eosinophilic vasculitis in medium-sized vessels throughout the colon. The thrombosis was improved after prednisolone and azathioprine were given. The results of all autoimmune tests, including those for anti-neutrophil cytoplasmic antibodies, were all negative except the elevation of serum immunoglobulin E (680 kU/L [normal, <25 kU/L]). CONCLUSION: Although the patient failed to meet the criteria of the Churg-Strauss syndrome, this case may represent an atypical localized variant of eosinophilic vasculitis of the gastrointestinal tract. Immunosuppressant therapy should be considered after surgery.

"Idiopathic Eosinophilic Vasculitis": Another Side of Hypereosinophilic Syndrome? A Comprehensive Analysis of 117 Cases in Asthma-Free Patients.

BACKGROUND: The absence of asthma may rule out a diagnosis of eosinophilic granulomatosis with polyangiitis in patients with hypereosinophilic syndrome (HES) and features of vasculitis. OBJECTIVE: To describe eosinophilic vasculitis (EoV) as a possible manifestation of HES in asthma-free patients. METHODS: We screened our hospital database and the literature for patients with HES who met the following 4 criteria: (1) histopathological or clinical features of EoV (biopsy-proven vasculitis with predominant eosinophilic infiltration of the vessel wall and/or features of vasculitis with tissue and/or blood hypereosinophilia [absolute eosinophil count >1.5 G/L]); (2) no other obvious causes of reactive eosinophilia, organ damage, and vasculitis; (3) the absence of antineutrophil cytoplasmic antibodies; and (4) the absence of current asthma. RESULTS: Ten of our 83 (12%) asthma-free patients with HES and 107 additional cases in the literature met the criteria for EoV. After a critical analysis of the patients' clinical and laboratory characteristics and outcomes, we identified 41 cases of single-organ EoV (coronary arteritis, n = 29; temporal arteritis, n = 8; cerebral vasculitis, n = 4). Of the remaining 76 patients with EoV, the most frequent manifestations (>10%) were cutaneous vasculitis (56%), peripheral neuropathy (24%), thromboangiitis obliterans-like disease (16%), fever (13%), central nervous system involvement (13%), deep venous thrombosis (12%), and nonasthma lung manifestations (12%). Blood hypereosinophilia more than 1.5 G/L was observed in 79% of patients, and necrotizing vasculitis was observed in 44%. CONCLUSIONS: Our results suggest that idiopathic EoV (HES-associated vasculitis) can be classified as an eosinophilic-rich, necrotizing, systemic form of vasculitis that affects vessels of various sizes in asthma-free patients.

Recurrent Cutaneous Eosinophilic Vasculitis: A Rare Entity.

Recurrent cutaneous necrotizing eosinophilic vasculitis is a rare entity described by Chen et al. It has a benign course without any systemic involvement. However, often long-term treatment with systemic steroids is required. The exact etiopathogenesis remains unknown. We report a female patient, who presented with recurrent pruritic purpuric papules and plaques affecting different body parts of long duration. Disease was well controlled with low dose systemic steroids, but invariably recurred on its withdrawal. Histology revealed the features of eosinophilic vasculitis. Subsequent detailed evaluation ruled out systemic involvement, underlying diseases, and any precipitating factors. Hence, a diagnosis of recurrent cutaneous eosinophilic vasculitis was made. Patient showed excellent response to prednisolone, and on tapering the drug, it was found that she needed a maintenance dose of 5 mg/day. We did not come across any previous reports of recurrent cutaneous eosinophilic vasculitis from India.

Eosinophilic Skin Diseases: A Comprehensive Review.

Eosinophilic skin diseases, commonly termed as eosinophilic dermatoses, refer to a broad spectrum of skin diseases characterized by eosinophil infiltration and/or degranulation in skin lesions, with or without blood eosinophilia. The majority of eosinophilic dermatoses lie in the allergy-related group, including allergic drug eruption, urticaria, allergic contact dermatitis, atopic dermatitis, and eczema. Parasitic infestations, arthropod bites, and autoimmune blistering skin diseases such as bullous pemphigoid, are also common. Besides these, there are several rare types of eosinophilic dermatoses with unknown origin, in which eosinophil infiltration is a central component and affects specific tissue layers or adnexal structures of the skin, such as the dermis, subcutaneous fat, fascia, follicles, and cutaneous vessels. Some typical examples are eosinophilic cellulitis, granuloma faciale, eosinophilic pustular folliculitis, recurrent cutaneous eosinophilic vasculitis, and eosinophilic fasciitis. Although tissue eosinophilia is a common feature shared by these disorders, their clinical and pathological properties differ dramatically. Among these rare entities, eosinophilic pustular folliculitis may be associated with human immunodeficiency virus (HIV) infection or malignancies, and some other diseases, like eosinophilic fasciitis and eosinophilic cellulitis, may be associated with an underlying hematological disorder, while others are considered idiopathic. However, for most of these rare eosinophilic dermatoses, the causes and the pathogenic mechanisms remain largely unknown, and systemic, high-quality clinical investigations are needed for advances in better strategies for clinical diagnosis and treatment. Here, we present a comprehensive review on the etiology, pathogenesis, clinical features, and management of these rare entities, with an emphasis on recent advances and current consensus.

A Case of Recurrent Cutaneous Eosinophilic Vasculitis / 대한피부과학회지

Recurrent cutaneous eosinophilic vasculitis (RCEV) is a rare cutaneous disease characterized by necrotizing vasculitis of small vessels, with exclusive eosinophilic infiltration and minimal, or no leukocytoclasis. It presents with recurrent erythematous or purpuric papules or plaques, or angioedema associated with peripheral eosinophilia. This disease follows a benign chronic course without evidence of systemic involvement. Systemic corticosteroid therapy is effective but the disease commonly pursues a relapsing and remitting course. Herein, we report a case of RCEV that was effectively treated with systemic corticosteroids and dapson(R)(diaminodiphenylsulphone). Furthermore, the patient did not show a relapse of the disease in spite the tapering the systemic corticosteroid dose.