J Korean Rheum Assoc.  2008 Jun;15(2):153-158. 10.4078/jkra.2008.15.2.153.

Wegener's Granulomatosis Presenting as Pyoderma Gangrenosum

• Nam SJ
• Woo JH
• Choi SJ
• Ji JD
• Song GG
• Lee YH

• KMID: 1270232
• DOI: http://doi.org/10.4078/jkra.2008.15.2.153

Abstract

Wegener's granulomatosis is multi-systemic disease characterized by granulomatous necrotizing vasculitis and it usually affects upper and lower respiratory tracts. Cutaneous manifestation as an initial presentation is unusual and comprises about 15% of cases. The most frequent skin lesions are palpable purpura, papules, ulcerations, vesicles, subcutaneous nodules, necrotizing ulcerations. We report a patient with Wegener's granulomatosis who presents as a pyoderma gangrenosum.

Keyword

Wegener granulomatosis; Pyoderma gangrenosum; Skin Manifestations

Figure

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  Fig. 1. (A) There are hemorrhagic pustules and multiple deep ulcers surrounded by a purple border with mucous discharge on her lower legs. (B) The skin lesion have showed tendency of wax and wane following disease activity. This photograph shows improvement of skin lesion after administration of immunosuppressants.

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  Fig. 2. (A) Chest PA shows nodular density in right upper lobe. (B) Chest CT shows round nodule with central air density in right upper lobe.

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  Fig. 3. Lung biopsy (×400) shows vasculitis (arrow) with histiocyte aggregation and necrosis.

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  Fig. 4. Skin biopsy (×40) shows subcutaneous inflammation (arrow) and magnification of this area (inset, ×200) shows neutrophilic infiltration of subcutaneous tissue.

Reference

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