Oral and Lower Extremity Ulcers as the Initial Presentation of Granulomatosis with Polyangiitis

Abstract

Background: Granulomatosis with polyangiitis (GPA) is a small vessel vasculitis characterized by lung and kidney involvement. It is typically a disease of white females and has a poor prognosis with the average life expectancy of 5 months for a patient without treatment. Oral and skin ulcers are considered to be rare presentations.

Case: A 39-year-old black male presented to the hospital with oral and skin ulcers and was diagnosed with GPA based on the biopsies of both cutaneous lesions and kidney. He was started on rituximab with minimal improvement. Later he was admitted to the ICU and had plasmapheresis, and he gradually improved and was discharged home 8 days after admission.

Conclusion: GPA is an aggressive vascular disorder resulting in possible organ system damage and failure. The role of the sickle cell trait in this patient is undefined, but this combination of gender, race, and presenting symptoms in GPA is extremely unusual.

Figure 1

Ulcer on third and fifth toes (arrows).

Figure 2

CT imaging showing multiple irregular pulmonary nodules (arrow) at different levels. The morphology of these nodules favors infectious/inflammatory nodules more than malignancy. Vasculitis can also have this appearance.

Figure 3

Light microscopy of a renal biopsy with multiple cores of renal tissue, of which approximately 40% is represented by cortex. Eighteen glomeruli are present, none of which are globally sclerotic. In up to four glomeruli, there are segmental cellular crescents, associated with fibrinoid necrosis and rupture of the capillary loops. Intact glomeruli do not show significant endocapillary hypercellularity. The tubulointerstitial compartment is marked by tubular injury, with tubular dilatation and epithelial simplification. There is mild and patchy inflammatory infiltrate, mixed. Multiple red blood cell casts are present throughout the biopsy. Occasional neutrophilic casts are also seen. There are mild interstitial fibrosis and tubular atrophy which comprise approximately 20% of the cortex. Arteries are marked by mild intimal fibrosis. Arterioles do not show significant hyalinosis. Toluidine blue-stained sections contain four intact glomeruli.

Figure 4

This section is stained for fibrinogen and kappa and lambda light chains. There is focal and segmental staining of glomeruli with fibrinogen, in areas of crescents/fibrinoid necrosis (arrow). Kappa and lambda stain equally throughout the tubulointerstitium.

Figure 5

Predominantly central patchy and confluent ground-glass and interstitial opacities (arrows). This pattern is nonspecific but, given the patient's history, is favored to represent alveolar hemorrhage secondary to vasculitis.