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[Atypical presentation of Wegener disease in childhood].
- Source :
-
Journal des maladies vasculaires [J Mal Vasc] 2008 Dec; Vol. 33 (4-5), pp. 242-6. Date of Electronic Publication: 2008 Nov 20. - Publication Year :
- 2008
-
Abstract
- The pediatric forms of Wegener granulomatosis (WG) are rare. The clinical picture and the profile have specificities compared to those of adults. We report a case of a girl aged of four years and a half who presented initially with a clinical picture of Henoch Schönlein purpura. Physical examination revealed additionally to purpura, scabby lesions on the buttocks. The histopathological examination of a skin biopsy disclosed histiocyte infiltration. There were no Ig A deposits on direct immunofluorescence study. One year later, the diagnosis of WG was suspected, when the patient developed a respiratory problem related to left pulmonary infarction. Screening for thromboembolic factors was positive for antiphosphilipid antibodies. Diagnosis of WG was confirmed by the histopathological study lung tissue and a significant titre of serum ANCA. Blood tests failed to provide evidence of renal involvement. Cyclophosphamide and prednisolone therapy was administrated. A relapse occurred one year later on the controlateral lung; but no biological marker of disease activity could be detected.
- Subjects :
- Child, Preschool
Diagnosis, Differential
Female
Granulomatosis with Polyangiitis diagnostic imaging
Granulomatosis with Polyangiitis pathology
Humans
Inflammation pathology
Lung Abscess pathology
Radiography, Thoracic
Tomography, X-Ray Computed
Granulomatosis with Polyangiitis diagnosis
Lung pathology
Subjects
Details
- Language :
- French
- ISSN :
- 0398-0499
- Volume :
- 33
- Issue :
- 4-5
- Database :
- MEDLINE
- Journal :
- Journal des maladies vasculaires
- Publication Type :
- Academic Journal
- Accession number :
- 19022597
- Full Text :
- https://doi.org/10.1016/j.jmv.2008.09.007