Journal of Clinical and Aesthetic Dermatology

OCT 2017

An evidence-based, peer-reviewed journal for practicing clinicians in the field of dermatology

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42 JCAD JOURNAL OF CLINICAL AND AESTHETIC DERMATOLOGY October 2017 • Volume 10 • Number 10 C A S E R E P O R T CONCLUSION Wegener's granulomatosis, now known as granulomatosis with polyangitis, is a chronic small vessel vasculitis that can present atypically as pyoderma gangrenosum. GPA is an important diagnosis to investigate with ANCA confirmatory testing in subjects with lesions resembling pyoderma gangrenosum, as this might be the presenting symptom of systemic GPA. Sustained remission is the goal for treatment in GPA. The current treatment of choice is glucocorticoid therapy with either rituximab or cyclophosphamide. REFERENCES 1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11. 2. Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. Arthritis Rheum. 2011;63(4):863–864. 3. Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum. 1990;33(8):1101–1107. 4. Finkielman JD, Merkel PA, Schroeder D, et al. Antiproteinase 3 antineutrophil cytoplasmic antibodies and disease activity in Wegener granulomatosis. Ann Intern Med. 2007;147(9):611– 619. 5. Reinhold-Keller E, Beuge N, Latza U, et al. An interdisciplinary approach to the care of patients with Wegener's granulomatosis: long-term outcome in 155 patients. Arthritis Rheum. 2000;43(5):1021–1032. Erratum in: Arthritis Rheum. 2000 Oct;43(10):2379. 6. Finkielman JD, Lee AS, Hummel AM, et al. ANCA are detectable in nearly all patients with active severe Wegener's Granulomatosis. Am J Med. 2007;120:643.e9–e14. 7. Daoud MS, Gibson LE, DeRemee RA, et al. Cutaneous Wegener's granulomatosis: clinical, histopathologic, and immunopathologic features of thirty patients. J Am Acad Dermatol. 1994;31(4):605–612. 8. Gibson LE, Daoud MS, Muller SA, Perry HO. Malignant pyodermas revisited. Mayo Clin Proc. 1997;72(8):734–736. 9. Handfield-Jones SE, Parker SC, Fenton DA, et al. Wegener's Granulomatosis Presenting as Pyoderma Gangrenosum. Clin Exp Dermatol. 1992;17:197– 200. 10. Reed WB, Jensen AK, Konwaler BE, Hunter D. The cutaneous manifestations in Wegener's granulomatosis. Acta Derm Venereol. 1963;43:250– 264. 11. Zycinska K, Wardyn K, Zielonka TM, Nitsch- Osuch A, Smolarczyk R. Cutaneous changes: an initial manifestation of pulmonary Wegener's granulomatosis. Adv Exp Med Biol. 2013;755:307– 310. 12. Jacob SE, Martin LK, Kerdel FA. Cutaneous Wegener's granulomatosis (malignant pyoderma) in a patient with Crohn's disease. Int J Dermatol. 2003;42(11):896–898. 13. Jiménez-Gallo D, Albarrán-Planelles C, Linares- Barrios M, et al. Pyoderma gangrenosum and Wegener granulomatosis-like syndrome induced by cocaine. Clin Exp Dermatol. 2013;38(8):878–882. 14. Stone JH, Merkel PA, Spiera R, Seo P, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363(3):221–232. 15. Jones RB, Tervaert JW, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med. 2010;363(3):211–220. 16. Wegener's Granulomatosis Etanercept Trial (WGET) Research Group. Etanercept plus standard therapy for Wegener's granulomatosis. N Engl J Med. 2005;352(4):351–361. 17. Wygoda A, Rutkowski T, Składowski K, Hejduk B. Low dose radiotherapy as an effective treatment in a patient with solitary Wegener's granulomatosis resistant to systemic treatment—case report. Contemp Oncol (Pozn). 2013;17(1):107–111. 18. Martinez V, Cohen P, Pagnoux C, Vinzio S, et al. Intravenous immunoglobulins for relapses of systemic vasculitides associated with antineutrophil cytoplasmic autoantibodies: results of a multicenter, prospective, open-label study of twenty-two patients. Arthritis Rheum. 2008;58(1):308–317. 19. Walsh M, Merkel PA, Peh CA, et al. Plasma exchange and glucocorticoid dosing in the treatment of anti- neutrophil cytoplasm antibody associated vasculitis (PEXIVAS): protocol for a randomized controlled trial. Trials. 2013;14:73. JCAD FIGURE 6. Kappa stain, negative FIGURE 4. Acid-fast bacilli stain, negative FIGURE 7. Lambda stain, negative FIGURE 5. Fite stain, negative

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