Multiple Cutaneous Manifestations in ANCA-Positive Eosinophilic Granulomatosis with Polyangiitis before and after Biologic Therapy: Clinical and Histopathologic Characterization of a Paradigmatic Case

Giorgia Carnicelli; Alvise Sernicola; Vito Gomes; Giulia Cundari; Stefania Trasarti; Roberta Priori; Teresa Grieco

doi:10.3390/jcm11247429

Multiple Cutaneous Manifestations in ANCA-Positive Eosinophilic Granulomatosis with Polyangiitis before and after Biologic Therapy: Clinical and Histopathologic Characterization of a Paradigmatic Case

J Clin Med. 2022 Dec 15;11(24):7429. doi: 10.3390/jcm11247429.

Authors

Giorgia Carnicelli¹, Alvise Sernicola², Vito Gomes³, Giulia Cundari⁴, Stefania Trasarti⁵, Roberta Priori⁶, Teresa Grieco²

Affiliations

¹ Department of Radiology, Humanitas Research Hospital IRCCS, Rozzano, 20089 Milan, Italy.
² Dermatology Unit, Department of Clinical Internal Anesthesiological and Cardiovascular Sciences, "Sapienza" University of Rome, 00161 Rome, Italy.
³ Department of Anatomy and Pathology, Ospedale San Filippo Neri, 00135 Rome, Italy.
⁴ Department of Radiological Sciences, Oncology and Pathology, "Sapienza" University of Rome, 00161 Rome, Italy.
⁵ Department of Cellular Biotechnology and Hematology, "Sapienza" University of Rome, 00161 Rome, Italy.
⁶ Rheumatology Unit, Department of Applied Clinical and Medical Therapy, "Sapienza" University of Rome, 00161 Rome, Italy.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare immune-mediated vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCAs). Having systemic and possibly severe involvement, a prompt recognition of its clinical features is crucial to achieve favorable patient outcomes. Although cutaneous manifestations represent key elements, these still remain poorly characterized. We report a case of ANCA-positive EGPA presenting with palpable purpura, livedo reticularis, and pemphigoid-like lesions that was successfully treated with glucocorticoid therapy and rituximab. This report portrays the evolution of cutaneous lesions in ANCA-positive EGPA and demonstrates how dermatologic signs may represent indicators of active disease, allowing for timely diagnosis and for the monitoring of disease activity during treatment.

Keywords: ANCA; cutaneous manifestation; eosinophilic granulomatosis with polyangiitis; rituximab; vasculitis.

Publication types

Case Reports

Grants and funding

This research received no external funding.