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Clinical Problem Solving: Pathology
October 20, 2008

Pathology Quiz Case 1: Diagnosis

Author Affiliations


Arch Otolaryngol Head Neck Surg. 2008;134(10):1118-1119. doi:10.1001/archotol.134.10.1118-a

Diagnosis: Wegener granulomatosis (WG)

Wegener granulomatosis is an uncommon systemic vasculitis that typically involves the upper airways, lungs, and kidneys, but any organ can be involved, including the skin. Approximately 15% of patients will develop skin lesions at some point during the course of the disease.1The most common cutaneous presentation of WG is characterized by palpable purpura and necrotic ulceration, which can appear at the same time or after signs of the disease have occurred in other organs, such as the lungs and kidneys. According to Comfere et al,1there is no specific site for cutaneous lesions in WG. The most common cutaneous histopathologic presentation of WG is leukocytoclastic vasculitis; however, granulomatous inflammation with or without vasculitis and palisaded extravascular granulomas may also occur.1Serologic testing, including measurement of the antineutrophil cytoplasmic antibody (c-ANCA) titer, is a useful tool to detect WG, and positive c-ANCA findings correlate well with the diagnosis of WG.2,3