An unusual concurrent dermatological and neurological manifestation in an elderly woman with eosinophilic granulomatosis with polyangiitis: a case report and literature review

Abstract

: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disease, occurs commonly in middle-aged females. EGPA involves multiple systems and hallmarked by small and medium vessel entanglement. Concurrent basal ganglia lacunar infarctions (BLI) and peripheral nervous (PN) manifestation rarely reported in dermatological co-morbidity. We encountered an 81-year-old female with controlled hypertension, presented at the Neurology department with an antecedent ten years history of tuberculosis and asthma, and five days of complaints of left limb weakness accompanied by bilateral feet numbness associated with abdominal ache. Two weeks before, she had attended the dermatology clinic for lower limb purpuric rashes, which not resolved at the presentation. Examination and series of investigations revealed eosinophilia, negative anti-neutrophil cytoplasmic antibodies (ANCA), mild anemia secondary to gastrointestinal involvement, and purpuric rash. Further, peripheral polyradiculoneuropathy noted. Radiographically, paraventricular, and bilateral basal ganglia lacunar infarction and cerebral atrophy documented. Nasosinusitis, multiple lung fibrous, and sclerotic foci, and aortic regurgitation observed. On a clinical evaluation basis, the patient managed with corticosteroids and supportive symptomatic treatment, the 7th day’s improvement observed. To the authors’ knowledge, this is the oldest EGPA patient reported with co-occurrence of nervous system and cutaneous manifestation. Further, the patient had a unique presentation of the occurrence of both BLI and PN findings. Yet, the cardiac manifestation of aortic valve regurgitation rather than mitral valve regurgitation. Through case-reports, when EGPA nervous and dermatological manifestation co-occurs, the likelihood of encountering negative ANCA result >70% yet this necessitate vast future researches.