Eosinophilic leukocytoclastic vasculitis – a spectrum ranging from Wells’ syndrome to Churg-Strauss syndrome?

Ratzinger, Gudrun; Zankl, Julia; Eisendle, Klaus; Zelger, Bernhard

doi:10.1684/ejd.2014.2411

Cited by 14 publications

(8 citation statements)

References 36 publications

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“…Associations between WS and other eosinophilic diseases and the possibility of its progression to CSS (eosinophilic granulomatosis with polyangiitis) have been discussed, as CSS shows similar blood (elevated IgE, eosinophilia, elevated eosinophilic cationic protein) and histologic findings. 5 , 6 , 7 Our patient presented with characteristic signs of WS but also suffered from asthma, which was indicative of CSS. Importantly, no signs of vasculitis could be detected in the histopathology, and the patient did not fulfill the American College of Rheumatology criteria for the diagnosis of CSS.…”

Section: Discussionmentioning

confidence: 70%

Eosinophilic cellulitis (Wells syndrome) successfully treated with mepolizumab

et al. 2018

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Section: Discussionmentioning

confidence: 70%

Eosinophilic cellulitis (Wells syndrome) successfully treated with mepolizumab

et al. 2018

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“…Due to its toxic effects, the latter causes structural alteration (denaturation) of collagen fibers and local tissue destruction . Because of potential overlaps with and transition to hypereosinophilic syndrome, eosinophilic leukocytoclastic vasculitis, and Churg‐Strauss syndrome, some authors have proposed Wells syndrome to be part of a continuous spectrum of (hyper‐)eosinophilic disorders . Considering the potential association with malignant or systemic autoimmune diseases, close follow‐up is warranted .…”

Section: Pathogenetic Aspectsmentioning

confidence: 99%

Wells syndrome

Weins

Biedermann

Weiss

et al. 2016

J Deutsche Derma Gesell

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Summary 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.

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“…The hypocomplementemic variant of urticarial vasculitis may be an early manifestations of lupus erythematosus, while the normocomplementemic variant usually appears as cutaneous single-organ vasculitis. Eosinophil-rich leukocytoclastic vasculitis 9 may show flame figures, and there is overlap with both Wells syndrome and eosinophilic granulomatosis with polyangiitis (EGPA). In Henoch-Sch€ onlein purpura, 10 the characteristic pattern of palpable purpura on the lower legs is associated with rheumatic, renal (haematuria and proteinuria) and gastrointestinal involvement (abdominal pain) and deposition of IgA around postcapillary venules (IgA vasculitis).…”

Section: Small Vessel Vasculitides (Svv)mentioning

confidence: 99%

Cutaneous vasculitis

Ratzinger¹,

Zelger²,

Frischhut³

et al. 2021

Diagnostic Histopathology

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Cutaneous vasculitides are inflammatory diseases starting at and evolving around the blood vessels of the skin. They should be separated from occluding vasculopathies, where the pathological process predominantely starts in the blood. They can be graded according to their relationship to systemic vasculitides as a cutaneous component of a systemic vasculitis, as a skin-dominant variant of a systemic vasculitis or as a cutaneous single organ vasculitis. Diagnostic measures, therapy and prognoses depend greatly on this relationship. For diagnostic purposes, a classification based on histopathological features in asscociation with clinicopathological correlation seems most reliable. We differentiate according to vessel size (small-medium-large) and involved cell types (leukocytes, macrophages) as well as extravascular features (necroses, granulomas). Thus, the similarities and overlaps between different manifestations become apparent.

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Eosinophilic leukocytoclastic vasculitis – a spectrum ranging from Wells’ syndrome to Churg-Strauss syndrome?

Cited by 14 publications

References 36 publications

Eosinophilic cellulitis (Wells syndrome) successfully treated with mepolizumab

Eosinophilic cellulitis (Wells syndrome) successfully treated with mepolizumab

Wells syndrome

Cutaneous vasculitis

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