A 28-year-old woman with poorly controlled asthma presented with 1 week of a painful non-pruritic rash, 4 days of fevers and abdominal pain, and 1 day of a left foot drop. Examination revealed palpable purpura on the abdomen (Fig. 1), back, and lower extremities (Fig. 2). Respiratory wheezes, diffuse abdominal tenderness, and a left foot drop with 0/5 strength were present. Blood count with differential was remarkable for 56% eosinophils. A diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) was made.
Figure 1.
Palpable purpura on abdomen.
Figure 2.
Palpable purpura on right lateral aspect of ankle.
EGPA, formerly Churg-Strauss syndrome, is a rare, small- to medium-vessel vasculitis characterized by eosinophilic infiltration of organs with necrotizing granulomas.1 There are typically three phases: difficult-to-control asthma, followed by blood and tissue eosinophilia, and ultimately systemic necrotizing vasculitis. The vasculitic phase includes mononeuritis multiplex in 75% of cases.2 Dermatologic manifestations include tender subcutaneous nodules, maculopapular rash, and non-thrombocytopenic petechiae and palpable purpura.1
EGPA is highly steroid-responsive, with long-term survival of 90% over 20 years.3 After treatment with high-dose steroids was started, the patient’s symptoms improved markedly. She went on to receive azathioprine for maintenance therapy and physical therapy for her foot drop.
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Conflict of Interest
The authors declare that they do not have a conflict of interest.
References
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