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 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.     

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.     

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.     

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.     

Bullous Eosinophilic Cellulitis in a Child Treated with Dapsone

Abstract: Eosinophilic cellulitis, or Wells syndrome, is a rare but well‐described condition in which bullous lesions are uncommon, especially in childhood. We report a case of bullous eosinophilic cellulitis recalcitrant to steroid therapy in a 9‐year‐old boy who was successfully treated with oral dapsone.     

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: 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.     

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.     

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 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.     

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.)     

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.     

Eosinophilic dermatoses

Eosinophilic dermatoses are a heterogeneous group of diseases, characterized by an eosinophil‐rich infiltrate and/or degranulation of eosinophils. Blood eosinophilia may be an associated feature. Typical, albeit not specific histological findings include ‘flame figures’, which are caused by the accumulation of cationic proteins released by eosinophils and subsequent collagen denaturation. “Classic” eosinophilic dermatoses include eosinophilic cellulitis (Wells syndrome), granuloma faciale, eosinophilic fasciitis (Shulman syndrome) and eosinophilic folliculitis (Ofuji disease). In addition, there is a multitude of skin diseases that present with varying degrees of eosinophilic infiltration. These include atopic dermatitis, bullous pemphigoid, urticaria, allergic contact dermatitis, prurigo nodularis, arthropod bite reaction, parasitic infections, and drug hypersensitivity. Even though these disorders share a common characteristic (tissue eosinophilia), they differ greatly in their clinical presentation.     

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.     

Lenalidomide in the management of eosinophilic dermatosis of hematological malignancy

Eosinophilic dermatosis of hematological malignancy is a paraneoplastic skin eruption associated with chronic lymphocytic leukemia and other B‐cell malignancies. It clinically resembles an insect bite reaction and it can precede the symptoms of the hematological malignancy or be related to a more aggressive course. Different treatments have been proposed, but partial response and recurrence are frequent. Herein, we describe a case of eosinophilic dermatosis associated with mantle cell lymphoma with remission after lenalidomide therapy.     

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.     

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.