Eosinophilic granulomatosis with polyangiitis poses a significant diagnostic challenge due to its varied clinical presentation. Here, we present a case of a 59-year-old female with a history of asthma and sinusitis, who manifested with an extremely rare presentation of drastic tense blisters and haemorrhagic bullae alongside purpuric lesions and peripheral neuropathy. Examinations revealed eosinophilia, positive antineutrophil cytoplasmic antibody, and characteristic pathological findings with small-vessel vasculitis in the purpura. Treatment with glucocorticoids and cyclophosphamide led to rapid improvement in peripheral eosinophilia, skin manifestations, and motor neuron deficits. Although rare, our case underscores that bullous skin lesions should be recognised as a potential cutaneous hallmark of eosinophilic granulomatosis with polyangiitis to aid timely diagnosis, since prompt treatment initiation is crucial given the potential irreversible organ damage and poor prognosis of eosinophilic granulomatosis with polyangiitis.
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http://dx.doi.org/10.1093/mrcr/rxae056 | DOI Listing |
Int Forum Allergy Rhinol
January 2025
Department of Otorhinolaryngology, Mayo Clinic in Arizona, Scottsdale, Arizona, USA.
Background/aim: Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic disease associated with nasal polyposis. Multiple biologics are used for managing EGPA, including some approved for nasal polyps (NP). This study investigated real-world biologic prescription patterns for EGPA and their impact on NP and endoscopic sinus surgery (ESS) use.
View Article and Find Full Text PDFTher Adv Respir Dis
January 2025
Department of Chest Medicine, Taipei Veterans General Hospital, No. 201, Section 2, Shipai Road, Beitou District, Taipei City 11217, Taiwan.
Background: REMIT is the first real-world study of mepolizumab effectiveness in patients with severe asthma (SA) in Taiwan.
Objectives: The primary objective evaluated changes in clinically significant exacerbations (CSEs; defined as use of oral corticosteroids (OCS) or emergency department (ED) visits and/or hospitalizations) in the 12 months pre- and post-mepolizumab treatment. Secondary objectives assessed changes in the number of CSEs requiring ED visits/hospitalizations and daily maintenance OCS (mOCS) dosage 12 months pre- and post-mepolizumab treatment.
AJNR Am J Neuroradiol
January 2025
From the Department of Radiology (I.R., S.P., A.K., O.S.), Boston Medical Center, Boston University Chobanian & Avedisian School of Medicine, Boston, Massachusetts
Background And Purpose: Granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA) are the most common causes of chronic sinusitis from systemic granulomatous diseases. While both are small- to medium-sized vasculitis with necrotizing granulomas, they have different clinical courses and prognoses. High-density sinus opacification has been reported in allergic fungal sinusitis with eosinophilic infiltrates.
View Article and Find Full Text PDFJ Cutan Pathol
January 2025
Department of Dermatology, University of Virginia School of Medicine, Charlottesville, Virginia, USA.
Clin Rheumatol
January 2025
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, China.
Objective: To investigate the similarities and differences of clinical manifestations and long-term prognosis between eosinophilic gastroenteritis (EGE) and eosinophilic granulomatosis with polyangiitis (EGPA) complicating GI involvement (EGPA-GI).
Methods: Sixty-two EGE and 30 EGPA-GI patients were retrospectively enrolled in PUMCH from 2008 to 2023. Baseline clinical records were collected.
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