Abstract: TH-PO187

Be Brash: Name That Rash

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Kazerouninia, Amir, Baylor College of Medicine, Houston, Texas, United States
  • Afzal, Azka, Baylor College of Medicine, Houston, Texas, United States
  • Dave, Natasha Naresh, Baylor College of Medicine, Houston, Texas, United States
  • Raghavan, Rajeev, Baylor College of Medicine, Houston, Texas, United States
Background

Granulomatosis with polyangiitis (GPA), is a relatively rare, potentially fatal anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. It is characterized by necrotizing granulomatous inflammation and vasculitis, affecting predominantly small to medium vessels. Here, an uncommon manifestation of vasculitis—livedo racemosa—was not addressed and the patient suffered a stroke.

Methods

A 60-year-old woman with hypothyroidism presented to her PCP for rash, progressive weakness, stocking/glove numbness, and intermittent fevers. The rash was not described or addressed, but EMG was ordered. Within a week, she presented to the ED for the same complaints. She was febrile and studies were notable for leukocytosis, 1+ blood on urinalysis, anemia, and hypoalbuminemia. General medicine diagnosed polyneuropathy, began gabapentin, and discharged her from the ED.

Shortly thereafter, she re-presented to the ED with a left fronto-temporal hemorrhagic stroke. Renal was consulted for AKI and noticed a purplish, lace-like, mottled, reticulated rash on both legs, suggestive of livedo reticularis, but with irregularities within the thickness of the reticulations, more consistent with livedo racemosa. Family noted the rash had been present for several months.

Her urine had RBC casts, serum was positive for MPO-ANCA, and kidney biopsy showed interstitial granulomas and focal partial artery necrosis, all evidencing GPA. Given hemorrhagic stroke at an uncommon site, brain angiography was performed showing subtle luminal irregularities in the bilateral small cerebral vessels consistent with CNS vasculitis. Chest CT and EMG were consistent with pulmonary and PNS GPA, respectively. She was given pulse dose steroids and started weekly rituximab infusions. The patient’s kidney function improved somewhat, but dysarthria continues, with only about 60% of her speech understandable by family members.

Conclusion

Livedo racemosa is always due to a secondary disorder and warrants further evaluation. Multiple clinicians missed the importance of her rash; any livedo-like rash in the setting of active urinary sediment, nondiabetic polyneuropathy, intermittent fevers, leukocytosis, anemia, and hypoalbuminemia should be biopsied allowing for early diagnosis of systemic illnesses such as vasculitis. If identified early, GPA remission is possible with immunosuppressive therapy, and may have prevented this patient's hemorrhagic stroke.