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A Rare Case of Eosinophilic Granulomatosis with Polyangiitis Associated with Cryoglobulinemia Presenting with a Bullous Skin Eruption of the Lower Limbs

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is an antineutrophil cytoplasmic antibody- (ANCA-) associated small vessel vasculitis with multisystem involvement. It is characterized with asthma, eosinophilia, and renal and peripheral nervous system involvement. However, EGPA prese...

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Autores principales: Abeyaratne, D. D. K., Liyanapathirana, C., Fonseka, C. L., Wijekoon, P. W. M. C. S. B.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5822773/
https://www.ncbi.nlm.nih.gov/pubmed/29560002
http://dx.doi.org/10.1155/2018/3124281
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author Abeyaratne, D. D. K.
Liyanapathirana, C.
Fonseka, C. L.
Wijekoon, P. W. M. C. S. B.
author_facet Abeyaratne, D. D. K.
Liyanapathirana, C.
Fonseka, C. L.
Wijekoon, P. W. M. C. S. B.
author_sort Abeyaratne, D. D. K.
collection PubMed
description BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is an antineutrophil cytoplasmic antibody- (ANCA-) associated small vessel vasculitis with multisystem involvement. It is characterized with asthma, eosinophilia, and renal and peripheral nervous system involvement. However, EGPA presenting with bullous skin eruption is an uncommon dermatological manifestation. We report a rare case of EGPA overlapped with mixed essential cryoglobulinemia presenting with a bullous skin eruption. CASE PRESENTATION: A 49-year-old female presented with bilateral lower limb erythematous bullous rash with bilateral lower limb numbness. She had bilateral ankle edema with frothyuria and a recent onset wheeze. Blood investigations revealed a marked peripheral eosinophilia with positive P-ANCA. Skin biopsy was suggestive of leukocytoclastic vasculitis. She also had positive cryoglobulins with a high rheumatoid factor titre. The patient was diagnosed of having EGPA with overlapping mixed essential cryoglobulinemia. Her skin eruptions and systemic manifestations improved with prednisolone and cyclophosphamide therapy. CONCLUSION: EGPA can rarely present with a bullous skin eruption and may rarely associate with secondary cryoglobulinemia. Early recognition of these rare manifestations and prompt treatment would prevent further complications and death.
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spelling pubmed-58227732018-03-20 A Rare Case of Eosinophilic Granulomatosis with Polyangiitis Associated with Cryoglobulinemia Presenting with a Bullous Skin Eruption of the Lower Limbs Abeyaratne, D. D. K. Liyanapathirana, C. Fonseka, C. L. Wijekoon, P. W. M. C. S. B. Case Rep Med Case Report BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is an antineutrophil cytoplasmic antibody- (ANCA-) associated small vessel vasculitis with multisystem involvement. It is characterized with asthma, eosinophilia, and renal and peripheral nervous system involvement. However, EGPA presenting with bullous skin eruption is an uncommon dermatological manifestation. We report a rare case of EGPA overlapped with mixed essential cryoglobulinemia presenting with a bullous skin eruption. CASE PRESENTATION: A 49-year-old female presented with bilateral lower limb erythematous bullous rash with bilateral lower limb numbness. She had bilateral ankle edema with frothyuria and a recent onset wheeze. Blood investigations revealed a marked peripheral eosinophilia with positive P-ANCA. Skin biopsy was suggestive of leukocytoclastic vasculitis. She also had positive cryoglobulins with a high rheumatoid factor titre. The patient was diagnosed of having EGPA with overlapping mixed essential cryoglobulinemia. Her skin eruptions and systemic manifestations improved with prednisolone and cyclophosphamide therapy. CONCLUSION: EGPA can rarely present with a bullous skin eruption and may rarely associate with secondary cryoglobulinemia. Early recognition of these rare manifestations and prompt treatment would prevent further complications and death. Hindawi 2018-01-16 /pmc/articles/PMC5822773/ /pubmed/29560002 http://dx.doi.org/10.1155/2018/3124281 Text en Copyright © 2018 D. D. K. Abeyaratne et al. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Abeyaratne, D. D. K.
Liyanapathirana, C.
Fonseka, C. L.
Wijekoon, P. W. M. C. S. B.
A Rare Case of Eosinophilic Granulomatosis with Polyangiitis Associated with Cryoglobulinemia Presenting with a Bullous Skin Eruption of the Lower Limbs
title A Rare Case of Eosinophilic Granulomatosis with Polyangiitis Associated with Cryoglobulinemia Presenting with a Bullous Skin Eruption of the Lower Limbs
title_full A Rare Case of Eosinophilic Granulomatosis with Polyangiitis Associated with Cryoglobulinemia Presenting with a Bullous Skin Eruption of the Lower Limbs
title_fullStr A Rare Case of Eosinophilic Granulomatosis with Polyangiitis Associated with Cryoglobulinemia Presenting with a Bullous Skin Eruption of the Lower Limbs
title_full_unstemmed A Rare Case of Eosinophilic Granulomatosis with Polyangiitis Associated with Cryoglobulinemia Presenting with a Bullous Skin Eruption of the Lower Limbs
title_short A Rare Case of Eosinophilic Granulomatosis with Polyangiitis Associated with Cryoglobulinemia Presenting with a Bullous Skin Eruption of the Lower Limbs
title_sort rare case of eosinophilic granulomatosis with polyangiitis associated with cryoglobulinemia presenting with a bullous skin eruption of the lower limbs
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5822773/
https://www.ncbi.nlm.nih.gov/pubmed/29560002
http://dx.doi.org/10.1155/2018/3124281
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