Eosinophilic cellulitis successfully treated with Methotrexate

Eosinophilic cellulitis is a rare inflammatory skin disease of unknown aetiology, first described by George Wells in 1971 as a recurrent granulomatous dermatitis with eosinophilia. It is typically characterised by recurrent pruritic erythematous plaques, vesicles or bullae usually involving the extremities and trunk. Many cases of eosinophilic cellulitis are difficult to treat and traditionally require long course of oral steroids, resulting in steroid side effects. This case adds to the literature in that we report the use of methotrexate to treat eosinophilic cellulitis, as a safe and efficacious therapeutic alternative.     

Eosinophilic cellulitis of papulonodular presentation (Wells'' syndrome)

Eosinophilic cellulitis (Wells' syndrome) is a rare condition of unknown aetiopathogenesis and is characterized by erythemal plaques and a histological picture of eosinophilic infiltration of the dermis with 'flame' figures. Here we describe a patient with the papulonodular variant of eosinophilic cellulitis associated with an unusual hepatic dysfunction.     

Wells' syndrome: a clinical and histopathologic review of seven cases

Wells' syndrome, or eosinophilic cellulitis, is characterized clinically by an acute dermatitis resembling cellulitis, which evolves into violaceous plaques that resolve spontaneously without scarring. The histopathologic features are dynamic, starting with dermal edema and infiltration of eosinophils, the development of "flame figures," and finishing with the appearance of phagocytic histiocytes. We present the clinical and histopathologic features of seven cases of eosinophilic cellulitis.     

Hypereosinophilic syndrome with various skin lesions and juvenile temporal arteritis

Hypereosinophilic syndrome (HES) is a multisystem disease with a high mortality rate. It is characterized by peripheral blood eosinophilia and eosinophilic infiltration of the skin and many other organs. The commonest cutaneous features include erythematous pruritic maculopapules and nodules, angio-oedema or urticarial plaques. However, some case reports have indicated that eosinophilic cellulitis, cutaneous necrotizing eosinophilic vasculitis, Raynaud's phenomenon and digital gangrene may also occur as cutaneous features of HES. Juvenile temporal arteritis (JTA) of unknown cause is characterized by an asymptomatic nodule in the temporal artery area in young adults. Histologically, the lesion is characterized by a significant intimal thickening with moderate eosinophilic infiltrates, constriction or occlusion of the vascular lumen and absence of giant cells. We report a patient with HES presenting with eosinophilic cellulitis, Raynaud's phenomenon, digital gangrene and JTA. JTA may also be one of the features of HES.     

Bullous eosinophilic cellulitis succession with eosinophilic pustular folliculitis without eosinophilia

Eosinophilic cellulitis is characterized clinically by an acute dermatitis resembling cellulitis with unknown etiology. Eosinophilic pustular folliculitis is also a rare inflammatory dermatosis characterized by recurrent crops of erythematous follicular papulopustules that coalesce to form annular plaques with unclear etiopathogenesis. We describe a 20-year-old white male who had vesiculobullous and plaque-like lesions on the hands and feet and was diagnosed with bullous eosinophilic cellulitis clinically and histologically without any etiological agents. Following therapy with oral corticosteroid and oral tetracycline capsules, the lesions disappeared. After a 2-month asymptomatic period, the patient developed pruritic follicular papules and pustules on the lower and upper extremities and upper back. Stool examination revealed Gierdia intestinalis eggs. The patient had complete clearance with treatment of ornidazol for 2 weeks and indomethacin for 2 months. This is the first report of bullous eosinophilic cellulitis coexisting with eosinophilic pustular folliculitis without eosinophilia in the English published work.     

Nodular presentation of eosinophilic cellulitis (Wells'' syndrome)

Eosinophilic cellulitis is a rare condition of unknown aetiology. The classical presentation is of a tender or mildly pruritic cellulitis-like eruption, that has typical histology characterized by tissue eosinophilia, oedema and "flame" figures. Other reported clinical presentations include papular and nodular eruptions. It may be recurrent, and preceded at a variable time by a pruritic papular eruption. We describe a patient with the rare nodular variant of eosinophilic cellulitis affecting the palms of the hands, which occurred 2 years after a nonspecific pruritic papular eruption, without an obvious precipitant and in the absence of the more typical cellulitis-like plaques.     

Wells syndrome

A rare eosinophilic dermatosis, Wells syndrome, also referred to as eosinophilic cellulitis, is characterized by great clinical variability. Typical findings include infiltrated erythematous plaques arising on the extremities. Lesions initially resemble erysipelas/cellulitis, however, they do not improve with antibiotic treatment. Eosinophilic cellulitis is a diagnosis of exclusion that may only be made over the course of the disease, taking into account clinical and characteristic histological findings (flame figures). Although multiple potential triggers have been proposed, the exact etiology remains unresolved. Involvement of abnormal Th2 cells, IL‐5, and activated eosinophilic granulocytes suggest a nonspecific hypersensitivity response to exogenous or endogenous stimuli. Corticosteroids may have a beneficial effect on the chronic, recurrent course frequently observed. The disease is often self‐limiting, healing without sequelae. Given that transitions to hematological and oncological disorders have been observed, patients should be closely followed up.     

Wells syndrome

Wells syndrome, or eosinophilic cellulitis, is a rare disorder characterized by erythematous plaques evolving into dermal or subcutaneous masses. The histopathology shows degenerative collagen, histiocytes, and eosinophils. Although most cases of Wells syndrome occur in adults, we present a child with these clinical and histopathologic findings.     

虫咬后嗜酸性蜂窝织炎一例

患者,男,24岁。因左手背虫咬后出现水肿性红斑4个月就诊。皮损组织活检示真皮大量嗜酸性粒细胞浸润,呈现“火焰征”。诊断：嗜酸性蜂窝织炎。给予口服抗组胺药、外用糖皮质激素治疗。     

Eosinophilic cellulitis (Wells' syndrome) associated with colon carcinoma

Eosinophilic cellulitis (Wells' syndrome) is an inflammatory dermatosis characterized by marked eosinophilic infiltrates. Drugs and various infections are recognized causes of eosinophilic cellulitis. Eosinophilic cellulitis has been reported in non‐hematological malignancies in two patients with squamous cell carcinoma and one with nasopharyngeal carcinoma. We report the association of eosinophilic cellulitis with adenocarcinoma of the colon. Curative hemicolectomy led to a complete remission, suggesting that underlying malignancies can trigger eosinophilic cellulitis.     

Eosinophilic cellulitis and dermographism

A 26-year-old man presented with a history of intermittent erythematous plaques on his hands and legs. A peripheral blood eosinophilia was noted. Histopathologic examination showed numerous eosinophils and characteristic flame figures. The clinical presentation and histopathologic alterations are consistent with the diagnosis of Wells' syndrome, which is also known as eosinophilic cellulitis. Wells' syndrome is a rare condition of unclear etiology. We discuss its diagnosis and possible association with other conditions that manifest peripheral eosinophilia.     

Wells' syndrome

Eosinophilic cellulitis/Wells' syndrome is a rare dermatosis with erythematous, urticarial plaques that become more indurated and eventually have grey blue discoloration. The histopathology is distinctive, with a diffuse infiltrate composed predominantly of eosinophils but admixed with lymphocytes, histicytes and occasionally multinucleated histiocytes. There is dermal edema with so called "flame figures" that is composed of collagen focally enveloped with aggregates of eosinophilic granules. These collagen fibres may be surrounded by palisading histiocyes. The course is variable with waxing and waning and eventual spontaneous resolution.     

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.     

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 cellulitis as a cutaneous manifestation of idiopathic hypereosinophilic syndrome

Three patients with eosinophilic cellulitis associated with sustained peripheral blood eosinophilia of unidentified cause are reported. They also presented with diversities of extracutaneous symptoms such as bronchospasm, sensory polyneuropathies, epigastralgia, and gangrenous eosinophilic enteritis. These cases suggest that eosinophilic cellulitis can develop as a cutaneous manifestation of idiopathic hypereosinophilic syndrome.     

Eosinophilic fasciitis and eosinophilic cellulitis in a patient with abnormal circulating clonal T cells: increased production of interleukin 5 and inhibition by interferon alfa

Eosinophilic fasciitis (Shulman's syndrome) and eosinophilic cellulitis are part of a spectrum of diseases characterized by tissue and peripheral blood eosinophilia. Eosinophils are implicated directly in the lesional process that characterizes these conditions, because signs of eosinophil activation and degranulation are observed at the sites of tissue injury. The cause and pathogenesis of eosinophilic fasciitis and cellulitis are presently unclear. Herein, we report a patient manifesting rapidly progressive localized cutaneous induration of the arms and legs with eosinophilia, no signs of systemic sclerosis, and histopathologic features compatible with the diagnosis of eosinophilic fasciitis. Four years after the onset of eosinophilic fasciitis, the patient had recurrent episodes of eosinophilic cellulitis. Blood screening for clonal T-cell receptor gamma gene rearrangements revealed several amplified clonal populations of circulating T cells. Furthermore, in vitro analysis of cytokine production by the patient's peripheral blood mononuclear cells demonstrated strongly increased production of interleukin 5, the synthesis of which could be completely blocked by interferon (IFN)-alpha. The coexistence of eosinophilic fasciitis and cellulitis in a patient with an abnormal circulating T-cell clone and increased IL-5 production are unique and might be responsible for the eosinophilia and eosinophil-mediated tissue injury. Although not assessed in vivo in this patient, our in vitro data provide a rationale for the use of IFN-alpha in eosinophilic fasciitis and/or cellulitis.     

Wells' syndrome or eosinophilic cellulitis. Apropos of 2 cases. Review of the literature

In 1971, four cases of a new dermatosis were described by Wells, under the name of recurrent granulomatous dermatitis with eosinophilia. In 1978, eight additional cases were reported by Wells and Smith and three authors suggested a shorter title: eosinophilic cellulitis for this syndrome. Since then, four additional cases were published in the literature. We report here two additional cases. From these eighteen upto now published cases, there is no doubt that this dermatosis, as initially described by Wells, is a distinct entity. Clinical course is characterized by sudden eruption of large infiltrated, itchy and/or painful plaques. Blisters are often associated. During the two or three weeks following the initial rash, the inflammatory aspect disappears. Lesions become indurated, and may resemble morphea. Spontaneous resolution occurs after about six weeks. Recurrences are constantly observed. Histologic features are a striking eosinophilic infiltrate associated with eosinophilic deposits constituting flame figures. Blood eosinophilia is present in most cases. Etiology of this entity remains unknown.     

Eosinophilic panniculitis: a clinicopathologic study

Study of 18 patients with biopsy diagnoses of eosinophilic panniculitis revealed diverse patterns of systemic disease, including Wells' syndrome, vasculitis, atopy, and erythema nodosum as well as localized panniculitis. Significant associated diseases included psychiatric illness, 6 (drug dependency, 4); atopy, 5 (asthma, 3); malignancies, 5; immune complex vasculitis, 4; thyroid disease, 2; Wells' eosinophilic cellulitis, 2; glomerulonephritis and sarcoidosis, 1 each. The skin lesions varied from urticarial papules and plaques to purpura, pustules, and ulcerative lesions but always included a nodular subcutaneous component, frequently as a presenting complaint. Eosinophilic panniculitis is a non-specific finding that can signify localized disease, such as an insect bite or injection lipophagic granuloma in a drug-dependent patient, or systemic lymphoma or immune reactive disease. Eosinophilic panniculitis in erythema nodosum is perhaps its most confusing presentation.     

Eosinophilic cellulitis (Wells syndrome) in a pediatric patient: a case report and review of the literature

We report a 4-year-old boy who presented with multiple pruritic, annular, erythematous plaques on the lower extremities of 1 week's duration. Histopathology of an affected area revealed a dense dermal infiltrate of eosinophils and flame figures without evidence of vasculitis. A diagnosis of eosinophilic cellulitis (EC), or Wells syndrome, was made. The patient had an excellent response to topical and systemic steroids following 1 week of treatment. This case appeared to be idiopathic, as there was no cause identified such as arthropod bites or tinea infection. The patient's EC cleared and has not had a recurrence.     

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.