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.
Atopic eczema (AE) or atopic dermatitis (AD) is an inflammatory skin disease with involvement of genetic, immunological and environmental factors which are highly interconnected. 1,2 The heterogenic disease can be separated into different phenotypes and clinical presentations defined by the ethnicity, disease onset, disease severity, chronic vs acute, intrinsic vs extrinsic (IgE level), paediatric vs adult and inflammatory signature. [3][4][5] A common feature of all subtypes is a tremendous psychosocial burden for all patients with AE. 6 Prevalence varies by area and is reported to be 15-20% in children in Europe, persisting in up to 5-10% of adults. [7][8][9] Although severe cases are less abundant than mild or moderate disease pattern, 2% of affected children are severely suffering. 7,9 Therefore, AE remains to be a high and even increasing socio-economic burden in the United States and in Europe, 10,11 whereas slightly decreasing numbers were reported over the last few years in Japan. 12 Children often overcome atopic eczema, but set off on the so-called 'atopic march', that is begin a classic 'allergy career'. Scientifically, AE is a risk factor for the development of allergies. These are primarily type I allergies with clinical features such as hay fever and asthma. Allergies are increasingly becoming a widespread disease. Currently, almost every fourth person in Europe suffers from symptoms such as asthma or hay fever and the associated restrictions in everyday life or at work. For society, the reduced ability to perform at school, university and at work means great socio-economic damage.
Das Wells-Syndrom, auch als eosinophile Zellulitis bezeichnet, ist eine seltene, sporadisch auftretende eosinophile Dermatose mit polymorphem klinischem Bild. Als typisch gelten entzündliche Erytheme oder Plaques an den Extremitäten, die initial als Erysipel imponieren können, unter antimikrobieller Behandlung aber persistieren. Die eosinophile Zellulitis ist eine Ausschlussdiagnose, die in Zusammenschau von klinischem Befund und charakteristischem histologischem Bild (Flammenfiguren) nur im Verlauf gestellt werden kann. Mit einer Vielzahl möglicher Triggerfaktoren ist die Ätiologie nicht geklärt. Die Beteiligung abnormer Th2-Zellen, des Zytokins IL-5 und aktivierter eosinophiler Granulozyten lässt eine unspezifische Hypersensitivitätsreaktion auf exo- und endogene Stimuli vermuten. Die häufiger vorkommenden chronisch- rezidivierenden Formen lassen sich durch Glukokortikoide günstig beeinflussen. Der Verlauf ist meist selbstlimitierend, ohne Residuen. Aufgrund von Übergängen in hämatoonkologische Erkrankungen sind Verlaufskontrollen angezeigt.
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