Tense blisters and hemorrhagic bullae as the first manifestation of eosinophilic granulomatosis with polyangiitis

Mod Rheumatol Case Rep. 2024 Sep 16:rxae056. doi: 10.1093/mrcr/rxae056. Online ahead of print.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) 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 hemorrhagic bullae alongside purpuric lesions and peripheral neuropathy. Examinations revealed eosinophilia, positive anti-neutrophil 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 recognized as a potential cutaneous hallmark of EGPA to aid timely diagnosis, since prompt treatment initiation is crucial given the potential irreversible organ damage and poor prognosis of EGPA.

Keywords: anti-neutrophil cytoplasmic antibody; eosinophilic granulomatosis with polyangiitis; hemorrhagic bullae; skin rash; tense blisters.