Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis

Marques, Camila Carneiro; Fernandes, Elizabeth Leocadia; Miquelin, Gabriela Momente; Colferai, Mariana Morais Tavares

doi:10.1590/abd1806-4841.20175522

Cited by 13 publications

(10 citation statements)

References 5 publications

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“…Cutaneous alterations occur in approximately 70% of the cases and manifest as hemorrhagic lesions: palpable purpura, petechiae, ecchymosis, and hemorrhagic bullae in 48%; subcutaneous nodules, frequently located on the scalp or distributed bilaterally over the extensor surfaces of the extremities, in 30%; maculopapular eruption in 25%; and urticariform eruptions and livedo reticularis in 25% of cases [ 2 , 4 , 5 ].…”

Section: Discussionmentioning

confidence: 99%

“…Prednisone is the indicated treatment (60–100 mg/day) until no more disease activity is detected (eosinophilia, ESR, and p-ANCA), at which point a gradual reduction of the dosage is initiated. In cases of rapid worsening and involvement of multiple organs, pulse therapy with steroids and immunosuppression with cyclophosphamide should be used [ 2 , 7 ].…”

Section: Discussionmentioning

confidence: 99%

“…Churg-Strauss syndrome (CSS), or eosinophilic granulomatosis with polyangiitis, or allergic granulomatous angiitis, is a rare autoimmune disorder characterized by excess circulating tissue eosinophils and vasculitis, which affects the lung and skin. It is a primary vasculitis characterized by late onset of asthma, multisystem involvement, and eosinophilic vasculitis of small- and medium-sized vessels [ 1 , 2 ]. This syndrome was first described by Churg and Strauss in 1951.…”

Section: Introductionmentioning

confidence: 99%

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Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis: Exuberant Classic Clinical Picture of a Rare Disease

et al. 2018

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The authors present a classic case of Churg-Strauss syndrome with an exuberant clinical picture in a 34-year-old woman. She showed the following diagnostic criteria: asthma, polyneuropathy, rhinopathy, marked eosinophilia, positive p-ANCA with a perinuclear pattern, and skin histopathology results suggestive of vasculitis with eosinophils. There was a good response to prednisone, dexamethasone pulse therapy, and cyclophosphamide.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis: Exuberant Classic Clinical Picture of a Rare Disease

et al. 2018

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“…Extravascular necrotizing granulomas may also be found. 41,42 Approximately 50% of the patients with Wegener's granulomatosis have painful subcutaneous nodules and punched out ulcers. Histological sections show necrotizing granulomatous vasculitis with blood vessels at various phases of involvement.…”

Section: Miscellaneous Granulomatous Conditionsmentioning

confidence: 99%

Histopathological Spectrum of Granulomatous Skin Lesions: A Review

George¹

2019

SBV Journal of Basic, Clinical and Applied Health Science

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Background and objectives: Granulomatous dermatoses are the most frequently encountered dermatological disorders in tropical countries with leprosy and tuberculosis as the leading causes. Although granulomatous skin lesions are very prevalent in society today, dermatopathologists often face diagnostic challenges while reporting these lesions due to the similar histopathological features or tissue reaction patterns seen in these lesions with diverse etiologies. The purpose of this study is to review the various causes and patterns of granulomatous skin lesions and a sequential approach to diagnosis of individual lesions accurately. Materials and methods: A comprehensive search of all literature available on granulomatous skin diseases between 2000 and 2018 was conducted, using keywords such as "granulomatous dermatosis, " "cutaneous tuberculosis, " "leprosy, " and "histopathological study" in the databases such as PubMed, ProQuest, and Google Scholar. The articles that satisfied the criteria were included in the final review. Results: The available evidence from the existing systematic literature reviews and articles suggested that the various tissue reaction patterns observed in granulomatous skin lesions are classified as epithelioid, palisaded, suppurative, xanthogranulomatous, foreign body, and other granulomatous patterns. These tissue reaction patterns are caused by infections, foreign bodies, metabolites, chemicals, and malignancies. Conclusion: Granulomatous dermatoses are a heterogeneous group of skin disorders that are frequently encountered in diagnostic dermatopathology. The histomorphology of a granuloma has to be correlated with the clinical history and ancillary techniques including special stains, culture, and molecular methods are necessary to arrive at a definitive diagnosis.

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“…2 Formation of necrotizing granulomas is also seen in the vasculitic phase. 3 While eosinophilic vasculitis and urticaria are common findings in EGPA, 1,4 EN-like lesions are less commonly reported. The lack of eosinophils seen on histology further supports the proposed mechanism for EN as a hypersensitivity reaction to an unknown trigger, as opposed to the inflammatory and cytotoxic effects of eosinophils postulated for the other cutaneous manifestations.…”

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confidence: 99%

Three concurrent morphological presentations of eosinophilic granulomatosis with polyangiitis

Lui

Choi

2021

Int J Dermatology

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Three concurrent morphological presentations of eosinophilic granulomatosis with polyangiitisDear Editor,The cutaneous manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) are varied and include petechiae/purpura, urticaria, ulcers, and tender skin nodules. 1 Multiple concurrent morphologies, however, have been less reported.A 62-year-old Chinese female presented a rash over the lower legs, forearm, and neck for a 2-month duration. She also reported bilateral hand and feet numbness, blurring of vision,

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Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis

Cited by 13 publications

References 5 publications

Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis: Exuberant Classic Clinical Picture of a Rare Disease

Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis: Exuberant Classic Clinical Picture of a Rare Disease

Histopathological Spectrum of Granulomatous Skin Lesions: A Review

Three concurrent morphological presentations of eosinophilic granulomatosis with polyangiitis

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