Bullous eosinophilic cellulitis (Wells'' syndrome) associated with Churg–Strauss syndrome

We report a patient with Churg-Strauss syndrome (CSS) with asthma, eosinophilia, nasal polyposis and ANCA-associated multisystem vasculitis, who's skin eruption started with erythematous urticarial-plaques followed by haemorrhagic bullae. Histology of the plaques revealed 'flame figures' in the dermis with no granulomatous or vasculitic process, consistent with the diagnosis of eosinophilic cellulitis or Wells' syndrome. The association of CSS and Wells' syndrome observed in this patient may have a common pathogenesis. CSS may induce Wells' syndrome by an unknown factor.     

Eosinophile Zellulitis (Wells-Syndrom)

Eosinophilic cellulitis (Wells' syndrome) is a rare disorder characterized clinically by recurrent erythematous plaques resembling cellulitis and histologically by a dermal infiltrate of lymphocytes, eosinophils and eosinophil debris between collagen bundles, forming flame figures in typical cases. A 71-year-old woman with Wells' syndrome with blood and bone marrow eosinophilia showed a good response to dapsone. The level of eosinophil cationic protein (ECP) in serum was elevated. Immunophenotyping of peripheral T cells revealed an increased proportion of CD3+CD4+T cells. The patients' cultured peripheral lymphocytes spontaneously released significant amounts of interleukin 5 (IL-5), but not interleukin 4 (IL-4) or interferon gamma (IFN gamma). These findings suggest that activated T cells may be involved in the pathogenesis of blood and tissue eosinophilia in this patient.     

Wells' syndrome presenting as a noninfectious bullous cellulitis in a child

Wells' syndrome is a rare disease that is even more uncommon in childhood. This case report illustrates the potential devastating extent of the disease and highlights the unusual presentation of bullae in a child. It is imperative to consider Wells' syndrome in patients with presumed cellulitis and eosinophilia who fail to respond to antibiotics.     

Inflammatory skin diseases and hypereosinophilia

Among the dermatoses associated with tissue eosinophilia are pemphigus vegetans, bullous pemphigoid, granuloma facial and Wells' syndrome. Eosinophilic spongiosis can occur in the early stage of pemphigus. Pemphigus herpetiformis, a recently introduced term, does not represent a well defined entity but develops into either pemphigus vulgaris or pemphigus foliaceus. Granuloma facial is basically vasculitis. Eosinophilic cellulitis or Wells' syndrome shows numerous eosinophils which degranulate and through confluence of their granules form the so-called flame figures.     

Wells' syndrome (eosinophilic cellulitis): correlation between clinical activity, eosinophil levels, eosinophil cation protein and interleukin-5

Wells' syndrome (WS) (eosinophilic cellulitis) is characterized by the presence of oedematous skin lesions associated with eosinophilia of the tissues. It has recently been observed that in patients with this disease, increased eosinophil cation protein (ECP) and interleukin (IL) -5 can be detected in peripheral blood, with T lymphocytes that have mRNA for this lymphokine. We present a patient with WS in whom we found a close correlation between clinical activity, eosinophils in blood and bone marrow, and ECP and IL-5 levels in peripheral blood and tissues. We underline the major part played by IL-5 in this disease.     

Wells' syndrome associated with parvovirus in a 5-year old boy

Wells' syndrome, otherwise known as eosinophilic cellulitis, is a rare dermatosis seen more commonly in adults than in children. In this article, we present a 5-year-old Caucasian boy who initially presented with pruritic, erythematous macules and papules evolving to bullae formation. Subsequent histology confirmed diagnosis of Wells' syndrome, and additional blood work also demonstrated positive immunoglobulin (Ig)M and IgG for parvovirus. In many instances, the direct etiology of Wells' syndrome is unclear, but the link between parvoviral infection and development of Wells' syndrome could further support an additional cause.     

The histopathogenesis of the flame figure in Wells' syndrome based on five cases

Characteristics of Wells' syndrome are recurrent episodes of edema and erythema of sudden onset, often covering large areas of the skin. Microscopy shows marked eosinophilia and the presence of so-called flame figures. The flame figures have been considered to be either secondary to aggregates of expelled eosinophilic granules and disintergrating eosinophils, or foci of necrobiotic collagen. Our study indicates that the flame figure is secondary to disintegration of eosinophils and consists of aggregates of eosinophilic granules and nuclear fragments and not of necrobiotic collagen. We consider Wells' syndrome to be a distinctive clinical and histological reaction, which can be triggered by many different, mostly unknown factors.     

Wells' syndrome. Recurrent granulomatous dermatitis with eosinophilia.

Two cases of granulomatous dermatitis with eosinophilla (Wells' syndrome) are reported. With Wells' original four cases, these two cases define a distinctive dermatosis with onset as cellulitis and formation of solid edema and either final spontaneous resolution or resolution with steroid therapy. Microscopic study showed diffuse tissue eosinophilia and fibrinoid flame figures, evolution of associated focal necrobiosis, and formation of focal microgranulomas associated with eosinophils. Biopsy of muscle and fascia showed comparable fasciitis and eosinophilic myositis. Immunofluorescence in one case disclosed fibrin in the dermis and lgM, lgA, and C3 in the blood vessels of the muscle. Recurrences of the lesions often appeared to be related to drug administration or surgery.     

Cutaneous larva migrans associated with Löffler's syndrome

We describe a patient who was admitted to hospital after returning from a holiday in Thailand with coronary artery disease and ventricular tachycardia. As an incidental finding, the routine chest radiography showed migratory pulmonary infiltrates. A peripheral blood smear showed eosinophilia. Physical examination revealed multiple, slightly raised, erythematous, serpentine tracks on the buttocks. A clinical diagnosis of larva migrans was made, and the patient was treated with oral albendazole 400 mg on 5 consecutive days and a single dose of oral praziquantel 3600 mg. By the end of treatment, most of the cutaneous lesions, the pulmonary infiltrates and the peripheral eosinophilia had resolved. The final diagnosis was larva migrans associated with L?ffler's syndrome.     

Insect-bite-like Wells' syndrome in association with mantle-zone lymphoma

Wells' syndrome is a multifaceted dermatosis with a wide morphological spectrum, ranging from characteristic cellulitis-like erythema and wheals to an unusual presentation of vesicles and bullae. We describe a patient in whom Wells' syndrome presented as an insect-bite-like eruption and was associated with underlying mantle-cell lymphoma. We recommend meticulous investigation of patients diagnosed with Wells' syndrome manifesting as an insect-bite-like eruption.     

Photosensitivity: a possible cause for Wells' syndrome?

Wells' syndrome is a rare inflammatory dermatitis. To date, the cause is unknown but it has been associated with a variety of triggers. We present a case of Wells' syndrome with coexisting photosensitivity.     

Eosinophilic cellulitis (Wells' syndrome) in association with angioimmunoblastic lymphadenopathy

Eosinophilic cellulitis (Wells' syndrome) is an uncommon inflammatory disease with clinical polymorphism. It is often associated with infectious, allergic or myeloproliferative diseases; however, the exact aetiology is unknown. This report describes a rare case of eosinophilic cellulitis in association with angioimmunoblastic lymphadenopathy. The typical skin findings of Wells' syndrome disappeared completely following chemotherapy and autologous stem cell transplantation.     

Eosinophilic panniculitis associated with chronic recurrent parotitis

A 37-year-old woman presented with recurrent erythema nodosum-like lesions on her arms and legs that were preceded by chronic recurrent parotitis. Microscopic examination revealed a mixed lobular and septal panniculitis with many eosinophils and multiple eosinophilic flame figures similar to those seen in Wells' syndrome. The dermis was not involved. The classification, differential diagnosis, and possible pathomechanisms of eosinophilic panniculitis are discussed.     

Eosinophilic cellulitis (Wells' syndrome): histologic and clinical features in arthropod bite reactions

Five patients are described with the clinical and histopathologic picture, including flame figures, of eosinophilic cellulitis (Wells' syndrome). Two of them had documented tick bites in the center of these expanding annular lesions, and the histologic picture showed the diagnostic flame figures of Wells' syndrome (eosinophilic cellulitis). A third patient had a clinical picture suggestive of a tick bite reaction but stated she was stung by a small garden bee at the involved site. The fourth patient removed a spider from the site of a spider bite, and this nodule also histopathologically was identical to that of eosinophilic cellulitis. Our fifth patient presented with papular urticaria of flea bites. We suggest that the characteristic flame figures of eosinophilic cellulitis (Wells' syndrome) are not diagnostic of a specific disease entity but rather a striking and peculiar histopathologic response to multiple factors of which arthropod bites (ticks, bees, fleas, and spiders) represent one definite etiology.     

皮损内注射复方倍他米松治疗Wells综合征一例

报告1例61岁女性患者,右前臂结节、斑块伴瘙痒1年。皮损组织病理示:真皮中下层见大量淋巴细胞及嗜酸粒细胞浸润,部分胶原束内可见嗜酸粒细胞脱颗粒形成"火焰"现象。诊断:Wells综合征(嗜酸性蜂窝织炎)。皮损内注射糖皮质激素治疗3个月后皮损消退,随访6个月未见复发。     

Hypereosinophile Dermatitis

BACKGROUND: The idiopathic hypereosinophilic syndrome is a rare systemic disease characterized by blood and tissue eosinophilia of unknown etiology, in which multiple organs may be affected. In hypereosinophilic dermatitis the only affected organ besides the blood is the skin. PATIENTS: We present a series of seven patients with hypereosinophilic dermatitis who were treated in our hospital between 2002 and 2003. RESULTS: All patients initially showed characteristic, therapy-resistant, polymorphic skin lesions, presenting with a combination of erythematous, pruritic and urticarial papules and plaques. All had blood eosinophilia without evidence of allergic, parasitic or other causes. The histology showed tissue eosinophilia only in half of the cases; the other histological findings were non-specific. We observed a good response to therapy with systemic corticosteroids, dapsone and light therapy, applied as UVA-1 irradiation or as shower photochemotherapy. CONCLUSIONS: The diagnosis "hypereosinophilic dermatitis" should be based primarily on the characteristic clinical picture together with "idiopathic" peripheral eosinophilia, whereas the histological findings are not always indicative. Because of the multiplicity of possible differential diagnoses and the often non-revealing histology, we assume that the diagnosis "hypereosinophilic dermatitis" is often overlooked.     

嗜酸性粒细胞增多综合征1例

患者男,78岁。全身出现丘疹,伴瘙痒2年余,出现红斑,伴脱屑半年余,既往体健。外周血和骨髓嗜酸性粒细胞增多,组织病理改变符合嗜酸性粒细胞增多综合征。予糖皮质激素、抗感染和支持对症治疗20d,痊愈。     

Eosinophilic arteritis of the scalp

BACKGROUND: The occurrence of recurrent peripheral arterial thromboses together with a blood eosinophilia generally suggests an occlusive vascular disease or a systemic vasculitis.CASE REPORT: In a 31-year-old woman with a 15-year history of severe smoking and a blood eosinophilia from 1,200 to 2,500/mm(3), we observed recurrent attacks of pruritus and urticaria and recurrent lesions of eosinophilic thromboangiitis of hypodermal medium-sized elastic arteries of the scalp. In spite of the persistent eosinophilia, the evolution was spontaneously regressive and no other clinical or biological sign occurred within a follow-up time of 3 years.DISCUSSION: In Buerger's disease (thromboangiitis obliterans) and in most systemic vasculitis, especially in Churg-Strauss syndrome, the first lesions may be inflammatory thromboses of the extra-cranial scalp arteries. The diagnosis of an eosinophilic arteritis of the scalp may only be considered if the examination of the other peripheral vessels is normal and if the course of the disease is benign, without any treatment, in spite of a persistent blood eosinophilia. This clinico-pathological presentation should be considered as a distinct entity.     

Onset of disseminated Bloch-Sulzberger incontinentia pigmenti]

The case report of a 2-week-old girl suffering from incontinentia pigmenti (Bloch-Sulzberger Syndrome) with disseminated urticarial erythemas, papules and vesicles at the onset is presented. Histological examination showed eosinophilic spongiosis. The peripheral blood eosinophilia and the development of typical lesions led to the diagnosis of incontinentia pigmenti. Eosinophilic spongiosis and peripheral blood eosinophilia are important symptoms in the diagnosis of atypical incontinentia pigmenti.