Wegener's granulomatosis : Case report and literature review

Wegener's granulomatosis (WG) is a necrotizing granulomatous vasculitis usually affecting the upper and lower respiratory tracts and kidneys. Any organ system can be affected by the pathologic process, which remains an etiologic enigma. Limited forms of the disease are recognized in which few e...

Full description

Saved in:
Bibliographic Details
Published in:Pediatric dermatology 1998-09, Vol.15 (5), p.352-356
Main Authors: STEIN, S. L, MILLER, L. C, KONNIKOV, N
Format: Article
Language:English
Subjects:
Biological and medical sciences
Child
Granulomatosis with Polyangiitis - diagnosis
Granulomatosis with Polyangiitis - pathology
Humans
Male
Medical sciences
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Skin - pathology
Citations: Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Wegener's granulomatosis (WG) is a necrotizing granulomatous vasculitis usually affecting the upper and lower respiratory tracts and kidneys. Any organ system can be affected by the pathologic process, which remains an etiologic enigma. Limited forms of the disease are recognized in which few extrapulmonary and no renal lesions occur. Cutaneous manifestations occur in 40% to 50% of patients with WG (1). Early diagnosis is imperative because treatment regimens reduce morbidity and mortality in this potentially fatal disease. We report WG in a 10-year-old boy whose upper respiratory tract symptoms began at age 8 years. The case is illustrative of the difficulties that can be encountered in attempting to make this diagnosis. Skin is an easily accessible organ for biopsy, thus an increasing familiarity with the typical biopsy specimen findings within a consistent clinical setting may aid in earlier diagnosis of WG.
ISSN:0736-8046
1525-1470
DOI:10.1046/j.1525-1470.1998.1998015352.x