Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), is a rare systemic vasculitis that classically manifests as necrotizing granulomas of the upper and lower respiratory tract, kidneys, and blood vessels; however, it may affect any organ system, including th...
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| Format: | Article |
| Language: | English |
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Wiley
2014-01-01
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| Series: | Case Reports in Rheumatology |
| Online Access: | http://dx.doi.org/10.1155/2014/850364 |
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| author | Basheer Tashtoush Roya Memarpour Yasmin Johnston Jose Ramirez |
| author_facet | Basheer Tashtoush Roya Memarpour Yasmin Johnston Jose Ramirez |
| author_sort | Basheer Tashtoush |
| collection | DOAJ |
| description | Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), is a rare systemic vasculitis that classically manifests as necrotizing granulomas of the upper and lower respiratory tract, kidneys, and blood vessels; however, it may affect any organ system, including the skin. Cutaneous manifestations occur in up to 45% of patients during the disease course, and are the presenting feature in 9% to 14% of patients. The most common skin lesion specific to GPA is palpable purpura, with the histopathologic correlate of leukocytoclastic vasculitis. However, a wide range of clinical and histologic features may be seen. We herein report a case of a previously healthy 52-year-old Caucasian man who presented with multiple progressively enlarging painful ulcers on his face, upper extremities, back, and abdomen over a two-month period. Skin biopsies revealed pyoderma gangrenosum-like features. Serological tests were positive for PR3/c-ANCA. Six months later, the patient developed recurrent episodes of sinusitis associated with nasal bleeds and eventually nasal septum perforation. Despite aggressive treatment with Cyclophosphamide and steroids over one year, the patient had persistent nonhealing large ulcers and developed multiple lung nodules with cavitary lesions. |
| format | Article |
| id | doaj-art-2db82aaa96734b70aa2168f444e2ff58 |
| institution | Kabale University |
| issn | 2090-6889 2090-6897 |
| language | English |
| publishDate | 2014-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Rheumatology |
| spelling | doaj-art-2db82aaa96734b70aa2168f444e2ff582025-02-03T01:09:10ZengWileyCase Reports in Rheumatology2090-68892090-68972014-01-01201410.1155/2014/850364850364Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with PolyangiitisBasheer Tashtoush0Roya Memarpour1Yasmin Johnston2Jose Ramirez3Department of Pulmonary and Critical Care Medicine, Cleveland Clinic Florida, 2950 Cleveland Clinic Boulevard, Weston, FL 33331, USADepartment of Pulmonary and Critical Care Medicine, Cleveland Clinic Florida, 2950 Cleveland Clinic Boulevard, Weston, FL 33331, USADepartment of Clinical Pathology, Cleveland Clinic Florida, 2950 Cleveland Clinic Boulevard, Weston, FL 33331, USADepartment of Pulmonary and Critical Care Medicine, Cleveland Clinic Florida, 2950 Cleveland Clinic Boulevard, Weston, FL 33331, USAGranulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), is a rare systemic vasculitis that classically manifests as necrotizing granulomas of the upper and lower respiratory tract, kidneys, and blood vessels; however, it may affect any organ system, including the skin. Cutaneous manifestations occur in up to 45% of patients during the disease course, and are the presenting feature in 9% to 14% of patients. The most common skin lesion specific to GPA is palpable purpura, with the histopathologic correlate of leukocytoclastic vasculitis. However, a wide range of clinical and histologic features may be seen. We herein report a case of a previously healthy 52-year-old Caucasian man who presented with multiple progressively enlarging painful ulcers on his face, upper extremities, back, and abdomen over a two-month period. Skin biopsies revealed pyoderma gangrenosum-like features. Serological tests were positive for PR3/c-ANCA. Six months later, the patient developed recurrent episodes of sinusitis associated with nasal bleeds and eventually nasal septum perforation. Despite aggressive treatment with Cyclophosphamide and steroids over one year, the patient had persistent nonhealing large ulcers and developed multiple lung nodules with cavitary lesions.http://dx.doi.org/10.1155/2014/850364 |
| spellingShingle | Basheer Tashtoush Roya Memarpour Yasmin Johnston Jose Ramirez Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis Case Reports in Rheumatology |
| title | Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis |
| title_full | Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis |
| title_fullStr | Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis |
| title_full_unstemmed | Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis |
| title_short | Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis |
| title_sort | large pyoderma gangrenosum like ulcers a rare presentation of granulomatosis with polyangiitis |
| url | http://dx.doi.org/10.1155/2014/850364 |
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