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.     

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.     

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 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.     

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.     

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.     

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 following the lines of Blaschko

Eosinophilic cellulitis is an inflammatory dermatosis of unknown aetiology. We describe a case following the lines of Blaschko, which may be the expression of cutaneous mosaicism, suggesting a possible underlying genetic predisposition for the development of Wells' syndrome.     

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.     

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.     

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: 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 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 (eosinophilic cellulitis)--case report and electron microscopic studies

We report a case of Wells' syndrome (eosinophilic cellulitis) with acute cutaneous swelling followed by indolent infiltration. The histopathology is characterized by a dense infiltrate of eosinophils and "flame figures" in the dermis. The electron microscopic findings are peculiar. This case responded well to oral antihistamines. The skin signs and symptoms disappeared completely two weeks later.     

Immunofluorescence identification of eosinophil granule major basic protein in the flame figures of Wells'' syndrome

An indirect immunofluorescence assay using formalin-fixed paraffin-embedded skin was performed on six biopsies from four patients with eosinophilic cellulitis (Wells' syndrome) to determine the extracellular localization of eosinophil granule major basic protein (MBP). Serial sections from each biopsy were treated with either affinity chromatography-purified antihuman-MBP or staphylococcal protein A purified rabbit IgG (control material). There was striking extracellular fluorescence localized to flame figures, and intracellular staining of eosinophils in all sections treated with anti-MBP as compared with controls. The pattern of MBP extracellular staining corresponded to the configuration of each flame figure (as verified by counterstain of the same section with haematoxylin and eosin). These findings show that MBP can be used as a marker for determining eosinophil degranulation and, because MBP is localized to flame figures, they suggest that MBP may play a pathogenic role in Wells' syndrome.     

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.     

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.