A Rare Cause of Palpable Purpura: Eosinophilic Granulomatosis with Polyangiitis
Eosinophilic granulomatosis with polyangiitis is a systemic vasculitis that affects small to medium vessels, belonging to the spectrum of antineutrophil cytoplasm antibody-associated vasculitis. It is a rare disease that presents with vasculitis, asthma and other eosinophilic symptoms. We present the case of a 67-year-old male, with a palpable purpura affecting the superior and inferior limbs, and abdomen, for the past month. The patient complained of chronic cough for the past year, which aggravated in the last month. Complementary studies allowed the diagnosis of eosinophilic granulomatosis with polyangiitis and the patient was treated with prednisolone until remission followed by azathioprine as a maintenance therapy. After one year of follow-up no recurrence was seen.
Jennette JC. Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Clin Exp Nephrol. 2013; 17:603-6.
Pereira N, Amaro C. Vasculites na infância. Rev Soc Port Dermatol Venereol. 2012; 0:174-80.
Churg J, Strauss l. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. 1951; 27:277-301.
Vaglio A, Casazza I, Grasselli C, Corradi D, Sinico RA, Buzio C. Churg-Strauss syndrome. Kidney Int.
Cuchacovich R, Justiniano M, Espinoza LR. Churg–Strauss syndrome associated with leukotriene receptor
antagonists (LTRA). Clin Rheumatol. 2007; 26:1769-71.
Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, et al. Churg-Strauss syndrome. Autoimmun Rev. 2015; 14:341-8.
Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A, et al. HLADRB4 as a genetic risk factor
for Churg–Strauss syndrome. Arthritis Rheum. 2007; 56:3159-66.
Radice A, Bianchi L, Sinico RA. Anti-neutrophil cytoplasmic autoantibodies: methodological aspects and clinical significance in systemic vasculitis. Autoimmun Rev. 2013;12:487-95.
Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990; 33:1094-100.
Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med. 2015; 26:545-53.
Grayson PC, Monach PA, Pagnoux C, Cuthbertson D, Carette S, Hoffman GS, et al. Value of commonly measured laboratory tests as biomarkers of disease activity and predictors of relapse in eosinophilic granulomatosis with polyangiitis. Rheumatology. 2015; 54:1351-9.
Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P; French Vasculitis Study Group (FVSG). The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine. 2011; 90:19-27.0
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