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

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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Publication Details

Journal
Modern Rheumatology Case Reports
Published
2024-09-15
DOI
https://doi.org/10.1093/mrcr/rxae056
Primary Topic
Vasculitis and related conditions
Type
article
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article

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

Jun Kikuchi, Hironari Hanaoka, Yuko Kaneko, Hiroto Horikawa et al.
Modern Rheumatology Case Reports
Vasculitis and related conditions
article

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

Jun Kikuchi, Hironari Hanaoka, Yuko Kaneko, Hiroto Horikawa, Yasushi Kondo, Mitsuhiro Akiyama, Hajime Inokuchi, Shuntaro Saito
article en

Abstract

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.

Modern Rheumatology Case ReportsVol. 9(1)
Keio University (JP)
No poverty
Openalex Percentile: Top 72%
Vasculitis and related conditions
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Tense blisters and haemorrhagic bullae as the first manifestation of eosinophilic granulomatosis with polyangiitis — Jun Kikuchi, Hironari Hanaoka, et al. · Modern Rheumatology Case Reports (2024) | TGRS Research Map | TGRS