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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40 JCAD JOURNAL OF CLINICAL AND AESTHETIC DERMATOLOGY October 2017 • Volume 10 • Number 10 C A S E R E P O R T G Granulomatosis with polyangitis (GPA), formerly known as Wegener's granulomatosis, is one of the antineutrophil cytoplasmic antibody (ANCA) vasculitides that primarily affects the small vessels. Other diseases in this category include microscopic polyangitis and eosinophilic granulomatosis with polyangitis, formerly known as Churg-Strauss. 1 First described in 1931 as a variant of polyarteritis nodosa, later advances in laboratory testing allowed for further classification of these diseases based on ANCA subtype. In 2011, Wegener's granulomatosis was renamed GPA. 2 We present an unusual case of GPA that was initially diagnosed as pyoderma gangrenosum. CASE PRESENTATION A 50-year-old Caucasian man with a past medical history of arthritis presented with a two-month history of progressive, round-oval, deep-seated ulcerations. At initial presentation, the patient developed a nodular abscess in the left groin that occurred after a scratch on his left arm. He presented to the emergency room (ER) and was treated with incision and drainage, which initially relieved the symptoms from the abscess. At that ER visit, a tissue culture was taken, which was later found positive for acid-fast bacilli (AFB) on smear. A few days later, the patient returned to the ER and was admitted to the hospital due to painful, enlarging ulcerations in multiple locations, at which point Dermatology was consulted. On physical exam, the patient had large, painful ulcerations on the left chest, left upper arm, and left malar and parotidomasseteric regions (Figures 1 and 2). The only associated symptom was intermittent fevers. His medical history was unremarkable except for a reported history of arthritis, and his social history did not reveal any drug abuse. Laboratory tests, including complete blood count, comprehensive metabolic panel, serum protein electrophoresis, urinalysis, urine drug screen, stool, C-reactive protein, rapid plasma reagin, hepatitis panel, and human immunodeficiency virus, were all negative. Likewise, urine, wound, and blood cultures were negative. However, cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) was positive (1:320). Skin biopsy showed wide areas of suppurative neutrophil-rich changes associated with an adjacent mononuclear cell infiltrate with abundant plasma cells and scattered multinucleated histiocytes (Figure 3). No evidence of a primary vasculitis was noted. Further staining was performed with AFB and Fite techniques (Figures 4 and 5), and these were negative for Mycobacteria and Nocardia respectively. In contrast to the incision and drainage specimen obtained in the ER, the AFB A B S T R A C T Granulomatosis with polyangitis, formerly known as Wegener's granulomatosis, is a multi-system vasculitis that has a variable clinical presentation. Although uncommon, cutaneous symptoms can be the initial presenting symptom of granulomatosis with polyangitis. We present an unusual case of pyoderma gangrenosum followed by a diagnosis of granulomatosis with polyangitis. We also provide a review of current literature on therapeutic options. KEYWORDS: Pyoderma gangrenosum, granulomatosis with polyangitis, Wegener's granulomatosis, vasculitis Pyoderma Gangrenosum- associated Granulomatosis with Polyangitis: A Case Report and Literature Review by MATTHEW B. ZARRAGA, DO; NICOLE SWENSON, DO; and BRAD GLICK, DO, MPH, FAOCD Dr. Zarraga is the chief dermatology resident at Wellington Regional Medical Center in Wellington, Florida; Dr. Swenson is a research fellow at the Center for Clinical and Cosmetic Research in Aventura, Florida; and Dr. Glick is the program director at Wellington Medical Center and Skin and Cancer Associates in Florida. J Clin Aesthet Dermatol. 2017;10(10):40–42 FUNDING: No funding was received for the preparation of this article. DISCLOSURES: The authors have no conflicts of interest relevant to the content of this article. CORRESONDENCE: Nicole Swenson, DO; Email:

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