Wells’ syndrome associated with eosinophilic granulomatosis with polyangiitis − A case report
Introduction. Wells` syndrome (eosinophilic cellulitis) is a chronic, recurrent disease characterized by episodes of erythematous and edematous plaques or nodules with occasional development of hemorrhagic bullae on the trunk and limbs. Eosinophilic granulomatosis with polyangiitis, formerly known a...
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Ministry of Defence of the Republic of Serbia, University of Defence, Belgrade
2021-01-01
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| Series: | Vojnosanitetski Pregled |
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| author | Radević Tatjana Kandolf-Sekulović Lidija Ristić Gorica G. Mijušković Željko P. |
| author_facet | Radević Tatjana Kandolf-Sekulović Lidija Ristić Gorica G. Mijušković Željko P. |
| author_sort | Radević Tatjana |
| collection | DOAJ |
| description | Introduction. Wells` syndrome (eosinophilic cellulitis) is a chronic, recurrent disease characterized by episodes of erythematous and edematous plaques or nodules with occasional development of hemorrhagic bullae on the trunk and limbs. Eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss syndrome, is a specific variant of the group of diseases characterized by necrotizing vasculitis of small to medium-sized blood vessels affecting multiple organ systems. The association of Wells` syndrome and eosinophilic granulomatosis with polyangiitis is very rare, and to our knowledge has been reported in only ten patients. Case report. We present a case of a 34-year-old woman with a 3-year history of periodical onset of erythematous plaques on the trunk and edematous plaques clinically resembling cellulitis on her lower limbs. The patient reported a one-year history of asthma, rhinosinusitis, and nasal polyposis. Skin biopsy revealed the presence of diffuse eosinophilic infiltrates in the dermis accompanied by characteristic “flame figures”. Further investigation showed peripheral blood eo-sinophilia (22.6%), bilateral maxillary sinusitis, presence of eosinophil infiltrates and microabscesses in the bron-chial wall, and pericapillary eosinophil infiltrates in the pulmonary interstitium shown by bronchoscopy and transbronchial biopsy, respectively. Treatment was started with methylprednisolone 0.5 mg/kg/day, and the dose was gradually tapered for the following twelve weeks. Complete remission of skin changes was achieved, but new lesions appeared in the past two years, which required repeated treatment. Conclusion. Association of these syndromes is unusual and may be based on the common pathogenetic background. We hypothesize that Wells` syndrome could be a stage preceding eosinophilic granulomatosis with polyangiitis, and that patients should be evaluated for eosinophilic granulomatosis with polyangiitis, since these two diseases overlap in clinical and laboratory findings. |
| format | Article |
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| institution | OA Journals |
| issn | 0042-8450 2406-0720 |
| language | English |
| publishDate | 2021-01-01 |
| publisher | Ministry of Defence of the Republic of Serbia, University of Defence, Belgrade |
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| series | Vojnosanitetski Pregled |
| spelling | doaj-art-a9dab1822faa4f8a8c3f3fa5489b944b2025-08-20T02:20:39ZengMinistry of Defence of the Republic of Serbia, University of Defence, BelgradeVojnosanitetski Pregled0042-84502406-07202021-01-0178667167510.2298/VSP190525114R0042-84501900114RWells’ syndrome associated with eosinophilic granulomatosis with polyangiitis − A case reportRadević Tatjana0https://orcid.org/0000-0001-9259-3031Kandolf-Sekulović Lidija1https://orcid.org/0000-0002-5221-5068Ristić Gorica G.2https://orcid.org/0000-0001-5760-492XMijušković Željko P.3https://orcid.org/0000-0003-0924-1719Military Medical Academy, Department of Dermatology and Venereology, Belgrade, Serbia + University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade, SerbiaMilitary Medical Academy, Department of Dermatology and Venereology, Belgrade, Serbia + University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade, SerbiaUniversity of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade, Serbia + Military Medical Academy, Department of Rheumatology, Belgrade, SerbiaMilitary Medical Academy, Department of Dermatology and Venereology, Belgrade, Serbia + University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade, SerbiaIntroduction. Wells` syndrome (eosinophilic cellulitis) is a chronic, recurrent disease characterized by episodes of erythematous and edematous plaques or nodules with occasional development of hemorrhagic bullae on the trunk and limbs. Eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss syndrome, is a specific variant of the group of diseases characterized by necrotizing vasculitis of small to medium-sized blood vessels affecting multiple organ systems. The association of Wells` syndrome and eosinophilic granulomatosis with polyangiitis is very rare, and to our knowledge has been reported in only ten patients. Case report. We present a case of a 34-year-old woman with a 3-year history of periodical onset of erythematous plaques on the trunk and edematous plaques clinically resembling cellulitis on her lower limbs. The patient reported a one-year history of asthma, rhinosinusitis, and nasal polyposis. Skin biopsy revealed the presence of diffuse eosinophilic infiltrates in the dermis accompanied by characteristic “flame figures”. Further investigation showed peripheral blood eo-sinophilia (22.6%), bilateral maxillary sinusitis, presence of eosinophil infiltrates and microabscesses in the bron-chial wall, and pericapillary eosinophil infiltrates in the pulmonary interstitium shown by bronchoscopy and transbronchial biopsy, respectively. Treatment was started with methylprednisolone 0.5 mg/kg/day, and the dose was gradually tapered for the following twelve weeks. Complete remission of skin changes was achieved, but new lesions appeared in the past two years, which required repeated treatment. Conclusion. Association of these syndromes is unusual and may be based on the common pathogenetic background. We hypothesize that Wells` syndrome could be a stage preceding eosinophilic granulomatosis with polyangiitis, and that patients should be evaluated for eosinophilic granulomatosis with polyangiitis, since these two diseases overlap in clinical and laboratory findings.http://www.doiserbia.nb.rs/img/doi/0042-8450/2021/0042-84501900114R.pdfcellulitiseosinophilic granulomatosis with polyangiitiseosinophilsflame figuresangiitisasthma |
| spellingShingle | Radević Tatjana Kandolf-Sekulović Lidija Ristić Gorica G. Mijušković Željko P. Wells’ syndrome associated with eosinophilic granulomatosis with polyangiitis − A case report Vojnosanitetski Pregled cellulitis eosinophilic granulomatosis with polyangiitis eosinophils flame figures angiitis asthma |
| title | Wells’ syndrome associated with eosinophilic granulomatosis with polyangiitis − A case report |
| title_full | Wells’ syndrome associated with eosinophilic granulomatosis with polyangiitis − A case report |
| title_fullStr | Wells’ syndrome associated with eosinophilic granulomatosis with polyangiitis − A case report |
| title_full_unstemmed | Wells’ syndrome associated with eosinophilic granulomatosis with polyangiitis − A case report |
| title_short | Wells’ syndrome associated with eosinophilic granulomatosis with polyangiitis − A case report |
| title_sort | wells syndrome associated with eosinophilic granulomatosis with polyangiitis a case report |
| topic | cellulitis eosinophilic granulomatosis with polyangiitis eosinophils flame figures angiitis asthma |
| url | http://www.doiserbia.nb.rs/img/doi/0042-8450/2021/0042-84501900114R.pdf |
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