Eosinophilic fasciitis leading to painless contractures

Huppke, Peter; Wilken, Bernd; Brockmann, Knut; Sattler, B; Hanefeld, F.

doi:10.1007/s00431-002-1038-1

Cited by 31 publications

(16 citation statements)

References 11 publications

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“…This clinical picture might be a phenotype of EF specific to childhood. 5 The cause of EF is unknown; however it has been reported in association with Borrelia burdogferi infection 15 and L-tryptophan ingestion. 16,17 It is unclear whether the history of taxidermy in our patient's bedroom had any potential relationship to her condition as tryptophan is not used in taxidermy.…”

Section: Discussionmentioning

confidence: 98%

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Eosinophilic fasciitis in a female child

Ortega‐Loayza

Merritt

Groben

et al. 2008

Journal of the American Academy of Dermatology

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Section: Discussionmentioning

confidence: 98%

“…To our knowledge, a total of 32 childhood cases have been reported in the English-language medical literature. [3][4][5][6][7][8][9][10][11][12][13] In the current report, we describe a 12-year-old girl with painless joint contractures and induration of the extremities consistent with EF. …”

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confidence: 93%

Eosinophilic fasciitis in a female child

Ortega‐Loayza

Merritt

Groben

et al. 2008

Journal of the American Academy of Dermatology

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“…2,9 Eosinophilic fasciitis is another condition that can resemble Wells' syndrome. Also seen more frequently among adults but reported in children, 18,19 it presents with acute onset of skin inflammation and resolves with hyperpigmentation and sclerodermalike skin changes. Unlike Wells' syndrome, eosinophilic fasciitis is characterized by arthritis as a prominent symptom, and it follows a more chronic course, with individual lesions requiring months or years to resolve.…”

Section: Discussionmentioning

confidence: 99%

Bullous “Cellulitis” With Eosinophilia: Case Report and Review of Wells' Syndrome in Childhood

Gilliam¹,

Bruckner

Howard³

et al. 2005

Pediatrics

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ABSTRACT. 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. W ells' syndrome, or eosinophilic cellulitis, is a rare, recurrent, inflammatory dermatosis of unknown pathogenesis. In 1971, Wells 1 described 4 patients with an acute pruritic dermatitis clinically resembling bacterial cellulitis but with histopathologic findings characterized by dermal eosinophilia, phagocytic histiocytes, and the presence of flame figures. He initially called this syndrome recurrent granulomatous dermatitis with eosinophilia but later simplified the name to eosinophilic cellulitis. 2 Wells' syndrome is seen more commonly among adults but has been observed among children. Some hypothesize that this syndrome may represent a hypersensitivity response to a circulating antigen. 2 Associated precipitants include insect bites, medication reactions, recent immunization, myeloproliferative disorders, malignancies, and infections. We describe a case of a young child with no identifiable triggering factors, and we review the evidence for evaluation and management of these pediatric cases. CASE REPORTA previously healthy, 1-year-old girl presented with acute onset of edematous erythematous plaques, with associated bullae, on her lower extremities and left arm (Fig 1). These lesions were pruritic but not painful, and the patient was afebrile. Her parents denied a history of insect bites, ingestion of medications, trauma, or other intercurrent illness. The patient's most recent immunizations had been received 3 months earlier. The patient did not have a history of asthma, and there was no family history of asthma or atopic disease.The patient was admitted with presumed bacterial cellulitis and was treated with intravenously administered oxacillin, without improvement. Her laboratory studies were significant for an elevated white blood cell count of 30 ϫ 10 9 cells per L, with peripheral eosinophilia of 48%. After the patient failed to respond to systemically administered antibiotics, examination of vesicle fluid was performed and revealed numerous eosinophils. Subsequently, the diagnosis of probable Wells' syndrome was made. Oral steroid therapy was started at 2 mg/kg, and the patient's cutaneous symptoms improved within 24 hours, leaving residual erythema and hyperpigmentation (Fig 2). Five days after the i...

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“…These are part of eosinophilic fibrosing disorders and share clinical and pathological features with EF but may include visceral disease [3, 27, 28]. Eosinophilia-myalgia syndrome in the late eighties was largely induced by ingestion of L -tryptophan [3, 29]. Some cases of EF are reported after L -tryptophan ingestion, suggesting an overlap in the pathogenesis [30, 31].…”

Section: Discussionmentioning

confidence: 99%

Eosinophilic Fasciitis 30 Years after – What Do We Really Know?

Antic¹,

2006

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Background: Eosinophilic fasciitis (EF) is a rare fibrosing disorder associated with peripheral eosinophilia and scleroderma-like induration of the distal extremities which affects substantially quality of life. Although the disease has been described 30 years ago, the etiology and pathomechanisms are still obscure, and consensus for therapy is lacking. Numerous case reports of patients with EF exist but series are scarce. Patients and Methods: Eleven patients with EF from the Department of Dermatology, Kantonsspital Aarau, the University Hospital Basel and the Outpatient Clinic of Dermatology, Triemli Hospital Zurich, Switzerland, were retrospectively studied. Results: In 4 patients the initial diagnosis was not recognized by the referring nondermatologists. The median age was 55 years, excluding the youngest patient ever diagnosed with EF (age = 1 year). All patients showed an induration of the skin, which led to painful contractures in the joints in 3 cases. All but 2 patients demonstrated edema. A slight predominance of the upper extremities was observed. Sclerodactyly was noticed in 1 patient. Three patients reported an initial trauma at the affected site. Two patients were tested positive for borreliosis. One patient subsequently developed aplastic anemia and Hashimoto thyroiditis. Visceral or extracutaneous involvement was absent. Eight patients had a full or partial recovery under corticosteroids whereas in 2, improvement could be achieved only with cyclosporine, azathioprine or cyclophosphamide. Conclusions: The diagnosis of EF can be established by clinical, laboratory and histological findings. In general, corticosteroids are highly efficacious in EF and only a minority of patients need other immunosuppressive or cytostatic drugs.

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Eosinophilic fasciitis leading to painless contractures

Abstract: our case shows that eosinophilic fasciitis can present without skin involvement and arthritis and therefore has to be regarded as a differential diagnosis of contractures in childhood. Pulsed steroid treatment was effective and without side-effects.

Cited by 31 publications

References 11 publications

Eosinophilic fasciitis in a female child

Eosinophilic fasciitis in a female child

Bullous “Cellulitis” With Eosinophilia: Case Report and Review of Wells' Syndrome in Childhood

Eosinophilic Fasciitis 30 Years after – What Do We Really Know?

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