Wells syndrome and its relationship to Churg–Strauss syndrome

Ratzinger, Gudrun; Zankl, Julia; Zelger, Bernhard

doi:10.1111/j.1365-4632.2012.05482.x

Cited by 26 publications

(25 citation statements)

References 27 publications

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“…There are similarities in distribution and appearance with eosinophil‐rich leukocytoclastic vasculitis and EGPA. Patients with Wells’ syndrome may develop more severe/obvious signs of vasculitis, just as patients with EGPA may over time develop patchy hemorrhagic lesions of Wells’ syndrome with flame figures . In some cases of Wells’ syndrome, not only are flame figures demarcated by eosinophils but also by palisading macrophages.…”

Section: Small Vessel Vasculitismentioning

confidence: 99%

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Vasculitic wheel – an algorithmic approach to cutaneous vasculitides

Ratzinger

Zelger

Carlson

et al. 2015

J Deutsche Derma Gesell

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SummaryBackground: Previous classifications of vasculitides suffer from several defects. First, classifications may follow different principles including clinicopathologic findings, etiology, pathogenesis, prognosis, or therapeutic options. Second, authors fail to distinguish between vasculitis and coagulopathy. Third, vasculitides are systemic diseases. Organ-specific variations make morphologic findings difficult to compare. Fourth, subtle changes are recognized in the skin, but may be asymptomatic in other organs. Our aim was to use the skin and subcutis as a model and the clinicopathologic correlation as the basic process for classification. Methods and Results:We use an algorithmic approach with pattern analysis, which allows for consistent reporting of microscopic findings. We first differentiate between small and medium vessel vasculitis. In the second step, we differentiate the subtypes of small (capillaries versus postcapillary venules) and medium-sized (arterioles/arteries versus veins) vessels. In the final step, we differentiate, according to the predominant cell type, into leukocytoclastic and/or granulomatous vasculitis. Conclusions: Starting from leukocytoclastic vasculitis as a central reaction pattern of cutaneous small/medium vessel vasculitides, its relations or variations may be arranged around it like spokes of a wheel around the hub. This may help establish some basic order in this rather complex realm of cutaneous vasculitides, leading to a better understanding in a complicated field.

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Section: Small Vessel Vasculitismentioning

confidence: 99%

“…Comment : EGPA is the exaggeration of the process that starts as eosinophilic leukocytoclastic vasculitis or leukocytoclastic vasculitis, type Wells’ syndrome .…”

Section: Small and Medium Vessel Vasculitismentioning

confidence: 99%

Vasculitic wheel – an algorithmic approach to cutaneous vasculitides

Ratzinger

Zelger

Carlson

et al. 2015

J Deutsche Derma Gesell

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“…Eine Vielzahl verschiedener Auslöser (Pharmaka, Infektionen, Kontaktallergene, UV-Licht) wurde in Einzelfallberichten diskutiert [4][5][6][7][8] . Aufgrund möglicher Assoziationen und mutmaßlicher Übergänge in andere Systemerkrankungen sehen einige Autoren die eosinophile Zellulitis als Teil des hypereosinophilen Erkrankungsspektrums [9][10][11][12][13] . Nicht abschließend geklärt ist jedoch, ob das Wells-Syndrom eine eigene Entität ist oder aber ein mikro-beziehungsweise makroskopisches Reaktionsmuster auf verschiedene Stimuli darstellt [ 2,6 ] .…”

Section: Weitere Klinische Untersuchungenunclassified

Refractory edema and erythema of the abdominal wall

Weins

Schneider

Gethöffer

et al. 2016

J Deutsche Derma Gesell

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HistoryAn 85-year-old female patient was referred to us with a suspected diagnosis of erysipelas of the lower abdomen, after seven days of systemic treatment with amoxicillin/clavulanic acid had not led to any improvement. The antibiotic had to be stopped prematurely due to angioedema of the tongue. The pruritic, slightly warm erythema with accompanying dysesthesia (sensation of stinging and distension) had developed over a period of four weeks (Figure 1 a); excoriations from scratching were considered possible portals of entry. An abscess was ruled out by ultrasound. Given multiple comorbidities (COPD, coronary heart disease, Clinical examination of the lower abdomen showed a poorly demarcated, warm erythema, measuring 20×15 cm, with accompanying lymphedema. In addition, there were isolated linear scratch marks (excoriations).

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“…Die eosinophilen Infiltrate verleihen den hämorrhagischen Makulae, Papeln oder Plaques oft einen bräunlichen Farbton [16]. Obgleich diese Befundkonstellation typisch ist, so ist sie doch nicht pathognomonisch.…”

Section: Anca-assoziierte Vaskulitidenunclassified

Kutane Symptome der Vaskulitiden

2015

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The skin is one of the organs most frequently involved in vasculitides. Cutaneous vasculitis may present (1) part of a systemic vasculitis (e.g., IgA vasculitis), (2) a skin-restricted or skin-dominant variant of the corresponding systemic vasculitis without clinically apparent visceral involvement (e.g., cutaneous IgA vasculitis), or (3) a vasculitis occurring exclusively in the skin (e.g., erythema elevatun diutinum). The clinical symptoms of vasculitides are markedly determined by the size of the predominantly affected blood vessels. Systemic polyarteritis nodosa is regarded as a medium vessel vasculitis and is associated with multiple skin symptoms: (1) vasculitis of digital arteries with ensuing digital infarction, (2) livedo racemosa and subcutaneous nodules, and (3) in some patients even purpura and hemorrhagic macules due to additional small vessel vasculitis. In contrast, in its skin-restricted entity (i.e., cutaneous polyarteritis nodosa), the predominant symptoms are subcutaneous nodules surrounded by livedo racemosa, often on the lower legs. Among small-vessel vasculitides palpable purpura with predilection for the legs is a nearly pathognomonic feature of immune complex vasculitis. Variations in clinical symptoms indicate additional pathophysiological mechanisms or different vascultides: (1) ANCA-associated vasculitides often also entail nodules or sometimes livedo, (2) cryoglobulinemic vasculitis additionally may present with necrosis at cold exposed areas and involvement of vessels of various size, (3) small vessel vasculitis associated with systemic lupus erythematosus or rheumatoid arthritis shows predilection for additional sites (e.g., nailfolds) and also involvement of vessels beyond postcapillary venules, (4) recurrent macular vasculitis in hypergammaglobulinemia also occurs on dependent parts, but shows numerous small hemorrhagic macules instead of palpable purpura, (5) erythema elevatum diutinum begins with brightly red to violaceous plaques at extensor sites, followed by fibrotic nodules. Consequently, cutaneous symptoms provide pivotal clues for further diagnosis and ensuing management of vasculitides.

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Wells syndrome and its relationship to Churg–Strauss syndrome

Abstract: We conclude that Wells syndrome could be the starting point of a pathogenetic process that might reach its maximum in Churg-Strauss syndrome. As a clinical consequence, patients with Wells syndrome should be evaluated and followed for Churg-Strauss syndrome.

Cited by 26 publications

References 27 publications

Vasculitic wheel – an algorithmic approach to cutaneous vasculitides

Vasculitic wheel – an algorithmic approach to cutaneous vasculitides

Refractory edema and erythema of the abdominal wall

Kutane Symptome der Vaskulitiden

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