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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41 JCAD JOURNAL OF CLINICAL AND AESTHETIC DERMATOLOGY October 2017 • Volume 10 • Number 10 C A S E R E P O R T obtained from the biopsy specimen showed no Mycobacteria. Gram, Grocott methenamine silver, and Giemsa were negative for bacteria or fungal elements while the Treponema stain was negative for spirochetes. Kappa and Lambda (Figures 6 and 7) were negative for light chain restriction, and the direct immunofluorescence was negative for immunoglobulin (Ig) G, IgA, IgM, C3, C5b-9, and fibrinogen deposition. The patient was diagnosed with Wegener's granulomatosis due to the elevated c-ANCA level, which is 99-percent specific for the disease. Also, other causes of pyoderma gangrenosum, such as leukemia, were ruled out. In the hospital, the patient was treated with intravenous corticosteroids and antibiotics, to which he responded well. DISCUSSION ANCA associated vasculitis can affect any caliber blood vessels; however, small vessels are most commonly affected. 1 According to the American College of Rheumatology, two of four criteria must be present for diagnosis of GPA, including nasal or oral inflammation, an abnormal chest radiograph, urinary sediment with red blood cells or casts, or granulomatous inflammation on biopsy. 3 Interestingly, a positive ANCA titer is not required for diagnosis; however, it will frequently be present. GPA is ANCA-positive for the neutrophil serine protease 3 (PR3); however, PR3-ANCA titers are not used to monitor response to treatment or predict relapse. 4 In confirmed cases of GPA, PR3-ANCA is reported to be positive in 84 to 92 percent of cases. 5,6 Organ systems most frequently involved with GPA are the upper and lower respiratory tracts and kidneys; however, the skin, heart, peripheral nervous system and eye can also be affected. 5 Clinical manifestations of GPA include rapidly progressive necrotizing glomerulonephritis and pulmonary hemorrhage with hemoptysis. 1 Cutaneous manifestations have variable morphologies, and might not be the initial presenting symptom. Palpable purpura in leukocytoclastic vasculitis or necrotizing ulcers similar to those seen in pyoderma gangrenosum might be seen. 7 The presence of skin lesions has been reported in 9 to 25 percent of cases, and in some reports are as high as 50 percent. 5,8,9 As in our case, presentation of pyoderma gangrenosum-like lesions preceeding the diagnosis of GPA have been reported in the literature. 10 However, the incidence of GPA presenting as PG-like lesions is infrequent, and has been reported in only one of 166 cases. 11 Biopsy alone is not sufficient for diagnosis; however, the histopathology of these lesions show characteristic granulomatous inflammation, neutrophil microabscesses, vasculitis, and necrosis. 12 Also, cocaine abuse has been reported to mimic rheumatologic disorders including GPA, and these presentations might be difficult to differentiate from autoimmune- mediated processes. 15 In the past decade, several advances in treatment regimens for GPA have improved outcomes. Prior to biologic therapy agents, the standard treatment regimen was remission induction with glucocorticoids and cyclophosphamide, trimethoprim/ sulfamethoxazole, and methotrexate. Cyclophosphamide for induction of remission is dosed 2mg/kg daily, can be increased up to 4mg/kg daily, and is given with a slow prednisone taper starting at 1mg/kg daily. 5 Rituximab, a monoclonal antibody against CD20, has shown efficacy in remission induction similar to that of cyclosphosphamide. In one study, 64 percent of patients treated with rituximab achieved remission at six months, as compared with 53 percent of patients treated with cyclophosphamide. 12 Another 12-month study showed similar sustained remission rates with either cyclophosphamide-azathioprine at 82 percent or rituximab at 76 percent. 15 Other treatment modalities are also reported in the literature. Etanercept, a monoclonal antibody against TNF-alpha, did not show efficacy in inducing or sustaining remission when added to the standard treatment regimen in subjects with GPA. 16 There are case reports in the literature of complete response to low- dose radiation therapy. A nasal and periorbital mass of GPA was successfully treated with 2 Gy of fractionated radiation therapy. 17 However, radiation therapy can only be used in limited disease. Intravenous immunoglobulin (IVIG) has been reported in successfully treating relapses of GPA, given as daily infusion for four consecutive days each month. One study reported a 59-percent remission rate, when added to stable doses of previous medications for GPA, such as methotrexate. IVIG is not used as a first-line agent, but is important to consider for relapses of GPA. 18 Current research is evaluating the efficacy of plasma exchange in treating ANCA-associated vasculitis, such as GPA. Plasma exchange is performed in addition to conventional glucocorticoid and cyclophosphamide therapy. 19 FIGURE 1. Left upper arm ulceration FIGURE 2. Left parotidomasseteric region ulceration FIGURE 3. Skin biopsy, hemotoxylin and eosin (H&E): areas of suppurative neutrophil-rich changes associated with an adjacent mononuclear cell infiltrate with abundant plasma cells and scattered multinucleated histiocytes

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