title: [Atypical presentation of Wegener disease in childhood].
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.
authors.affiliation: Service de pédiatrie, hôpital La Rabta, 1007 Tunis, Tunisia.
authors.forename: H
authors.initials: H
authors.lastname: Ben Turkia
authors.forename: N
authors.initials: N
authors.lastname: Amdouni
authors.forename: H
authors.initials: H
authors.lastname: Azzouz
authors.forename: N
authors.initials: N
authors.lastname: Tebib
authors.forename: M-S
authors.initials: MS
authors.lastname: Abdelmoula
authors.forename: F
authors.initials: F
authors.lastname: El Mazni
authors.forename: A
authors.initials: A
authors.lastname: Hamzaoui
authors.forename: M F
authors.initials: MF
authors.lastname: Ben Dridi
mesh_terms: D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D007249:Inflammation / Q000473:pathology; D008168:Lung / Q000473:pathology*; D008169:Lung Abscess / Q000473:pathology; D013902:Radiography, Thoracic; D014057:Tomography, X-Ray Computed