Churg-Strauss syndrome and Wells' syndrome: coincidence or pathogenetic association? A new case report (original) (raw)
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Wells’ syndrome associated with eosinophilic granulomatosis with polyangiitis − A case report
Vojnosanitetski pregled
Introduction. Wells` syndrome (eosinophilic cellulitis) is a chronic, recurrent disease characterized by episodes of erythematous and edematous plaques or nodules with occasional development of hemorrhagic bullae, on the trunk and limbs. Eosinophilic granulomatosis with polyangiitis formerly known as Churg-Strauss syndrome is a specific variant of the group of diseases characterized by necrotizing vasculitis of small to mediumsized blood vessels affecting multiple organ systems. The association of Wells` syndrome and eosinophilic granulomatosis with polyangiitis is very rare and according to our knowledge has been reported in only ten patients. Case report. We present a case of a 34year-old woman with a 3-year history of periodical onset of erythematous plaques on trunk and edematous plaque that clinically resembling cellulitis on her lower limbs. The patient reported a one-year history of asthma, rhinosinusitis and nasal polyposis. Skin biopsy revealed the presence of diffuse eosinophilic infiltrate in the dermis accompanied by characteristic "flame figures". Further investigation showed peripheral blood eosinophilia (22.6%), bilateral maxillary sinusitis, presence of eosinophil infiltrates and microabscesses in bronchial wall and pericapillary eosinophil infiltrates in lung shown by bronchoscopy and transbronchial biopsy, respectively. Treatment was started with methylprednisolone 0.5 mg/kg/day and the dose was gradually tapered for the following twelve weeks. Complete remission of skin changes was achieved but new lesions appeared in the last two years which required repeated treatment. Conclusion. Association of these syndromes is unusual and may be based on the common pathogenetic background. We hypothesize that Wells` syndrome could be prior stage of eosinophilic granulomatosis with polyangiitis and that patients should be evaluated for eosinophilic granulomatosis with polyangiitis since these two diseases overlap in clinical and laboratory findings.
Eosinophilic cellulitis (wells syndrome): a case report
International Journal of Research in Dermatology, 2021
Eosinophilic Cellulitis is also known as Wells syndrome is uncommon dermatitis, characterized by the infiltration of eosinophils in the dermis. The exact etiology of the disease is unknown. Clinically, it is highly varied but commonly the presentation is pruritic erythematous plaque. We report a case of one and half years old healthy boy who developed itchy bullae on the dorsum of his hand with multiple erythematous papules over his extremities that started immediately after his vaccines. Histopathological examination of the lesion showed infiltrate eosinophils with typical flame figures. The case was successfully treated with corticosteroid course. This report aims to present a case of eosinophilic cellulitis (Wells syndrome) triggered by the vaccine with a literature review of the disease. In conclusion, atypical presentation of any inflammatory disorder or that not responding to appropriate treatment should trigger suspicion of eosinophilic cellulitis. Up to now, systemic cortico...
Wells syndrome and its relationship to Churg-Strauss syndrome
International journal of dermatology, 2013
Wells syndrome has been described as an inflammatory disorder based on typical clinical appearance combined with the histopathological presence of eosinophilic infiltrates and flame figures in the absence of vasculitis. Churg-Strauss syndrome, on the other hand, is primarily a diffuse, necrotizing vasculitis but is also typically displaying eosinophils and flame figures. Despite several parallels, the present understanding of these two diseases excludes any pathogenetic relationship. We describe the clinical course and histopathological appearance of three patients who had initially been diagnosed with Wells syndrome that developed into Churg-Strauss syndrome during the course of their disease. The clinical presentation of all three patients led to the diagnosis of Wells syndrome by independent specialists. Histopathology showed an eosinophilic infiltrate and flame figures next to features of leukocytoclastic vasculitis. Detailed examination revealed asthma bronchiale and addi...
The Canadian journal of plastic surgery = Journal canadien de chirurgie plastique, 2012
Eosinophilic cellulitis (Wells' syndrome) is an inflammatory dermatitis that is often misdiagnosed as infectious cellulitis due to its similarity in presentation. Misdiagnosis leads to delay of correct treatment and inappropriate use of antibiotics. A case series of eosinophilic cellulitis and a literature review are presented. Patients with Wells' syndrome may present with a variety of nonspecific symptoms, such as fever, arthralgia and malaise, as well as myriad cutaneous lesions with associated erythema, presenting as blisters, bullae, papules and/or nodules. Several treatment modalities have been used to treat eosinophilic cellulitis and have been met with variable success rates; these include systemic corticosteroids, topical corticosteroids and antihistamines, with success rates of 91.7%, 50% and 25%, respectively. A high degree of clinical suspicion must be exercised to diagnose this rare condition. Cellulitis with an atypical presentation or not responding to appropr...
Unusual Presentation of Wells Syndrome: A Case Report
2017
Email: dr.shakoei@gmail.com Wells syndrome is an uncommon disease that typically presents as edematous erythematous plaques, usually preceded by burning or itching of the skin. Histopathological examination shows dense dermal eosinophilic infiltrates in an edematous dermis at the acute phase of lesions. Some of the identified triggering factors include infection, arthropod bites, hematological malignancies, thimerosal containing vaccines and drugs such as penicillin, lincomycin, tetracycline, minocycline and ampicillin. Here we describe a case of Wells syndrome in a 75-year-old woman that its outstanding feature was its large size. Although this case was resistant to our treatment, the condition improved spontaneously after several weeks without administering any other alternative treatments. On the other hand, despite its large size, this case had no identifiable trigger.
Bullous "Cellulitis" With Eosinophilia: Case Report and Review of Wells' Syndrome in Childhood
PEDIATRICS, 2005
A 1-year-old girl presented with acute onset of edematous erythematous plaques associated with bullae on her extremities and accompanied by peripheral eosinophilia. She was afebrile, and the skin lesions were pruritic but not tender. The patient was treated with intravenously administered antibiotics for presumed cellulitis, without improvement. However, the lesions responded rapidly to systemic steroid therapy. On the basis of lesional morphologic features, peripheral eosinophilia, and cutaneous histopathologic features, a diagnosis of Wells' syndrome was made. Wells' syndrome is extremely rare in childhood, with 27 pediatric cases reported in the literature. Because it is seen so infrequently, there are no specific guidelines for evaluation and management of Wells' syndrome among children. The diagnosis should be considered for children with presumed cellulitis and eosinophilia who fail to respond to antibiotics. Evaluation should include a directed history, physical examination, complete blood count, and stool testing for ova and parasites, to identify potential triggers. Treatment is with systemic steroid therapy unless disease is limited, in which case medium/high-potency topical steroids may be indicated. If systemic features are prominent or disease is chronic (lasting >6 months), then a referral to hematology/oncology should be considered.
Wells Syndrome with Multiorgan Involvement Mimicking Hypereosinophilic Syndrome
Case reports in dermatology, 2009
Eosinophil-associated diseases represent a spectrum of heterogeneous disorders, where blood and cutaneous eosinophilia is the most important feature and eosinophils are the principal cause of cutaneous lesions. These diseases show some similarities in the clinical features but also many distinctive characteristics [Saurat et al., Dermatologia e malattie sessualmente trasmesse, Milano, Masson, 2000]. Wells syndrome is one of these disorders and is an uncommon recurrent inflammatory dermatosis, rarely associated to signs and symptoms of multiple organ involvement [Arch Dermatol 2006;142:1157-1161]. Hypereosinophilic syndrome, in contrast, constitutes a group of idiopathic disorders characterized by blood eosinophilia for at least 6 months, associated with single or multiple organ system dysfunction [Arch Dermatol 2006;142:1157-1161]. Clinically atypical Wells syndrome with multiorgan involvement is reported here. A correct diagnosis is difficult in this case, but clinical and histopat...
Eosinophilic cellulitis (Wells' syndrome): a new case description
Journal of the European Academy of Dermatology and Venereology, 1999
Background Wells' syndrome was first described by Wells in 1971 as a recurrent granulomatous dermatitis with eosinophilia and was later named eosinophilic cellulitis. It is defined by the following criteria: (i) sudden onset of annular or circinate erythematous-edematous patches that rapidly evolve to morphea-like blue-slate-colored plaques; (ii) a histological picture usually characterized by the presence of 'flame figures'; (iii) non-constant blood hypereosinophilia.