[Atypical presentation of Wegener disease in childhood].
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.
Ben Turkia
H
H
Service de pédiatrie, hôpital La Rabta, 1007 Tunis, Tunisia.
Amdouni
N
N
Azzouz
H
H
Tebib
N
N
Abdelmoula
M-S
MS
El Mazni
F
F
Hamzaoui
A
A
Ben Dridi
M F
MF
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