Recurrent cutaneous necrotizing eosinophilic vasculitis: a case report and review of the literature
© Li et al.; licensee BioMed Central Ltd. 2013
Received: 20 June 2013
Accepted: 26 October 2013
Published: 7 November 2013
We report a case of recurrent cutaneous necrotizing eosinophilic vasculitis (RCNEV) in a 57-year-old male. The patient presented with papules and pruritus of the lower limbs of more than 1 month duration, and with angioedema and intensively pruritic, necrotizing lesions of the bilateral anterior tibias and feet for 2 weeks. Treatment with systemic corticosteroids was administered for 1 month, and resulted in a significant improvement. We also present a review of the pertinent literature and discuss the clinical features, histopathological features, and differentiation of RCNEV.
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KeywordsVasculitis eosinophilic Recurrent Ctaneous necrotizing Corticosteroid
Recurrent cutaneous necrotizing eosinophilic vasculitis is a rare disease, which has clinical features of annular urticarial plaques, pruritic purpuric papules, angioedema, long course, chronic relapsing process, and an absence of any features of the systemic disease. Histopathological findings of RCNEV show necrotizing vasculitis of dermal small vessels with prominent eosinophilic infiltration. RCNEV was first reported by Chen, in 1994, and to the best of our knowledge there are only five patients with RCNEV described in the literature. Peripheral blood eosinophilia is a feature of many diseases such as hypereosinophilic syndrome, Wells syndrome, Churg-Strauss syndrome, and eosinophilic fasciitis. Compared with the aforementioned diseases, RCNEV has distinct pathological features of fibrinoid degeneration on small vessel walls and necrotizing vasculitis of dermal small vessels with prominent eosinophilic infiltration. Here, we report a case of RCNEV in a 57-year-old male, whose diagnosis was made using clinical, histopathological, and laboratory analysis results, and who was treated using systemic corticosteroids.
The patient was initially treated with 1 mg/kg prednisone daily, compound glycyrrhizin 150 mg daily. Mupirocin ointment was applied to the necrotic lesions. Immediate improvements in clinical manifestations and inflammation were noted. After 2 days, intensive pruritus and angioedema decreased rapidly. After 7 days, pruritus and angioedema had disappeared, and laboratory examinations showed that white blood cell and eosinophil cell count had normalized. One month later, the necrotizing lesions healed, with some remaining as superficial scars. The dose of prednisone was then slowly decreased. All hematological and biochemical examinations were normal and no new lesions were noticed after 4 months of follow-up. As any attempts to discontinue prednisone always result in recurrence, the patient presently continues to take prednisone at a dose of 10 mg daily.
Chen et al. reported one male and two female patients diagnosed with RCNEV. The male patient, a 17-year-old in 1973, noted purpuric lesions on his feet and buttocks, which later involved his entire body. One of the two females was a 56-year-old woman in 1989, and presented with pruritic purpuric lesions of 6-month duration. Another woman, at the age of 18 years in 1967, presented with gingivitis, pruritic, erythematous, and purpuric papules, and angioedema of the hands. Skin biopsy specimens showed a common feature: necrotizing vasculitis with marked perivascular eosinophil infiltration. In 2000, Launay et al. reported one patient who was an 81-year-old woman in 1996 who presented with intensively itchy, infiltrating, necrotic purpuric lesions on the lower limbs for 2 weeks. Skin biopsy was performed on a necrotizing lesion which showed necrotizing vasculitis in the deep dermis with striking infiltration by eosinophils. In 2007, Tanglertsampan et al. reported a 53-year-old white male diagnosed with RCNEV. He was treated with indomethacin, with a favorable response.
The pathogenesis of RCNEV is not fully understood, but cytotoxic eosinophil granule proteins such as major basic proteins are found to deposit in the areas of blood vessels, suggesting that eosinophils mediate vascular damage in this disease process[3, 4]. Eosinophils release IL-5, C4, and platelet-activating factor, which may lead to increased vascular permeability and induce cutaneous lesions such as purpuric papules and angioedema[5, 6]. Although he had more than five years history of psoriasis, no evidence confirmed a correlation between psoriasis and RCNEV.
Clinicopathological features of HES, Wells syndrome, CSS and EF HES
Sex ratio (M:F)
HES can happen at any age, although it is more common in adults
Wells syndrome usually affects adults
CSS can present at any age, with the mean age of onset being 40 years
EF usually affects adults between 20 and 60 years
Skin rashes such as urticaria or angioedema
Urticaria, cellulitis, annular plaques vesiculo-bullous lesions and edema
Hypereosinophilia, asthma, pulmonary infiltrates, and clinical evidence of vasculitis
Similar to scleroderma or systemic sclerosis
Eosinophilic infiltration with few lymphocytes, perivascular infiltration in dermis region, but not true necrotizing vasculitis
Eosinophilic infiltrates and flame figures in the absence of vasculitis. perivascular eosinophilic infiltration in the dermis, but not true necrotizing vasculitis
Peripheral blood eosinophils increase significantly, and neutrophil-rich leukocytoclastic vasculitis and granulomatous
Numerous inflammatory infiltrations of lymphocytes and eosinophils within the fascia
Prednisone, hydroxyurea, chlorambucil and vincristine.
Corticosteroids, calcineurin inhibitors, griseofulvin, H1 antihistamines, cyclosporine, dapsone
Prednisolone, azathioprine and cyclophosphamide
More than 80% of HES patients survive five years or more
It tends to resolve in weeks or months, usually without scarring. It occasionally recurs. In these recurrent cases, it can take years to ultimately resolve
The mean five years mortality rate is 28%
The prognosis is usually good in the case of an early treatment if there is no visceral involvement
In our case, not only an exclusive clinical histopathological feature of fibrinoid degeneration of small vessel walls, but also necrotizing vasculitis of dermal small vessels with prominent infiltration of eosinophils strongly supported the diagnosis of RCNEV.
With regard to the treatment of RCNEV, systemic corticosteroids are most commonly selected because of their effectiveness. Immediate improvements in clinical manifestations and inflammation were noted in our patient following the administration of prednisone. However, some measures should be taken to avoid its side effects if it is used persistently. Compound glycyrrhizin can reduce the activity of T-lymphocyte subset and play an anti-inflammatory role. Mupirocin ointment was used to prevent or control local infection of necrotic lesions. This therapeutic regimen proved to be suitable in our patient, and we consequently obtained a good clinical outcome.
RCNEV is rare and has clinical and histopathological features, which are different to other conditions of hypereosinophilia and eosinophilic vasculitis. Systemic corticosteroid is very effective in the treatment of RCNEV.
Written informed consent was obtained from the patient for publication of this Case Report and any all accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Recurrent cutaneous necrotizing eosinophilic vasculitis
Anti-neutrophil cytoplasmic antibodies
We would like to thank Dr. Qun Liu in Qianfoshan Hospital, Shandong University for helping in histological analysis. We also wish to thank International Science Editing, Compuscript Ltd. For editing the manuscript.
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