Eosinophilic cellulitis associated with urticaria. A report of two cases

Two patients with the clinical and histopathologic findings of eosinophilic cellulitis are presented. In one patient there was a definite relationship between upper respiratory tract infection and flare-ups of eosinophilic cellulitis. This patient's condition was superimposed on a long history of chronic urticaria. In the other patient a relationship to recurrent sinusitis was possible but not definite. There were no other contributory underlying skin diseases in either patient. Eosinophilic cellulitis is not likely a disease entity as such but, instead, represents a severe urticarial hypersensitivity reaction to various stimuli. Collagen alteration by eosinophil granules results in flame figure formation and a granulomatous response, which may explain the prolonged persistence of the lesions.     

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.     

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.     

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.     

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.     

Wells' syndrome associated with idiopathic hypereosinophilic syndrome

Wells’ syndrome, or eosinophilic cellulitis, is characterized by recurrent cutaneous swellings which resemble acute bacterial cellulitis, and by distinctive histopathological changes. Skin lesions show dermal eosinophilic infiltration and the characteristic‘flame figures, which are composed of eosinophil major protein deposited on collagen bundles. The idiopathic hypereosinophilic syndrome is a multisystem disease with a high mortality rate. It is characterized by peripheral blood eosinophilia and eosinophilic infiltration of many organs, including the skin. The most common skin lesions are pruritic maculopapules and nodules over the trunk and limbs, with urticaria and angio-oedema. In contrast to Wells’ syndrome, the pathology of these skin lesions is non-specific with variable eosinophil infiltration. We report overlapping clinical and histopathological findings characteristic of both syndromes in one patient. Our data favour the hypothesis that both syndromes represent an abnormal eosinophilic response to a variety of underlying diseases or causative agents and thus are different expressions of one disease entity linked to theimmunobiology of eosinophils.     

Unexplained hypereosinophilia and the need for cytogenetic and molecular genetic analyses

BACKGROUND: Idiopathic hypereosinophilic syndrome (HES) is a diagnosis made after the exclusion of other causes of eosinophilia. However, differentiation of idiopathic HES from eosinophilic leukemia is sometimes difficult. In some cases, these diagnoses can be differentiated by cytogenetic or molecular findings, as illustrated in the patients described herein. OBSERVATIONS: We describe 3 patients with HES and associated pruritus; 1 patient also had recurrent lesions of eosinophilic cellulitis. All 3 patients were initially diagnosed as having idiopathic HES, but after evaluation and demonstration of molecular abnormalities, they were classified as having eosinophilic leukemia. CONCLUSIONS: Patients with a diagnosis of idiopathic HES should be evaluated for cytogenetic or molecular genetic abnormalities. These abnormalities can establish a diagnosis of chronic eosinophilic leukemia and may provide clues for emerging therapies.     

Eosinophilic Cellulitis in a Family

Eosinophilic cellulitis (Wells' syndrome) is characterized by recurrent episodes of nonpruritic, indurative, cutaneous swellings. Three cases of this syndrome occurred in two male siblings and their mother. In all three family members, skin lesions appeared early in infancy. Unusual clinical manifestations included eosinophilic pleural effusions and pericarditis.     

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.     

Well’s综合征1例

报告1例Well's综合征。患者女,31岁。四肢反复出现皮疹,伴瘙痒、疼痛1年余,加重1月。组织病理示:真皮内大量嗜酸性细胞浸润,并可见火焰现象。诊断为嗜酸性蜂窝织炎(Well's综合征)。     

Familial eosinophilic cellulitis, dysmorphic habitus, and mental retardation

Background: Eosinophilic cellulitis is a polymorphous, chronic disease characterized by eosinophil infiltration and granulomatous inflammation. Objective: Our purpose was to describe the clinical, histologic, and immunohistologic findings in three family members who have had eosinophilic cellulitis since childhood associated with mental retardation and abnormal body habitus. Methods: Family members were evaluated. Multiple skin biopsy specimens were obtained and examined after hematoxylin-and-eosin staining, by immunofluorescence and by electron microscopy. Blood specimens were analyzed by immunoassays for eosinophil granule proteins and eosinophil active cytokines. Results: Three short-statured, mentally retarded family members with abnormal body habitus in at least two generations had recurrent eosinophilic cellulitis. Peripheral blood and bone marrow eosinophilia was present. Plasma eosinophil granule major basic protein and eosinophil-derived neurotoxin levels were elevated with normal plasma eosinophil cationic protein levels. Eosinophil survival in culture was increased by patients’ plasma and was blocked with monoclonal interleukin-5 antibody. The level of plasma interleukin-5 was elevated. Lesional skin biopsy specimens showed massive staining for three eosinophil granule proteins. Electron microscopy showed eosinophil disruption. Conclusion: Eosinophilic cellulitis, mental retardation, and abnormal body habitus were likely inherited as a dominant syndrome in this family in which eosinophil involvement was striking. (J Am Acad Dermatol 1998;38:919-28.)     

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.     

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.     

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.     

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.     

Eosinophilic cellulitis presented with semicircular pattern

Eosinophilic cellulitis (Wells' syndrome) is a rare condition of unknown etiology and pathogenesis. It is characterized by erythematous plaques and a histological picture of dermal eosinophilic infiltration with "flame figures". The typical clinical presentation of eosinophilic cellulitis is mildly pruritic cellulite-like plaques. Urticarial, vesiculo-bullous, nodular and papulonodular variants were also reported. Herein, we describe a patient with annular and semicircular manifestations of eosinophilic cellulitis. It was treated successfully with low-dose cyclosporine A treatment.     

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.     

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.     

Eosinophilic Cellulitis (Wells'' Syndrome)

Two patients with eosinophilic cellulitis are reported and 22 additional cases from the literature are reviewed. Cutaneous lesions are variable in appearance and may be confused with cellulitis, urticaria, insect bites, or contact dermatitis. Microscopically, there is a dense dermal infiltrate of eosinophils. Subsequently, granulomatous features with characteristic "flame figures" become apparent. Peripheral eosinophilia is common. The etiology of eosinophilic cellulitis is unknown, although a hypersensitivity mechanism is suspected. Treatment with systemic corticosteroids is frequently effective, but the disease is often characterized by relapses that can occur for several years.     

Eosinophilic cellulitis: five cases

Five cases of eosinophilic cellulitis or Wells' syndrome are described. While only few children have been included in earlier reports, 4 of the 5 patients in the present paper were below 10 years of age, with the youngest being only 20 months when the disease started. One of the children developed hard and tender subcutaneous swellings on the scalp, the histology of which showed extensive subcutaneous necrotizing granulomas. Similar lesions have not been described previously in connection with eosinophilic cellulitis. Eosinophilic cellulitis may be called a rare disease. However, it is important that clinician and histopathologist are both acquainted with the pathological features of this condition, as the disease often responds readily to steroid therapy.