Strawberry gingivitis as the first presenting sign of wegener's granulomatosis: report of a case
© I. Holzapfel Publishers 2011
Received: 10 March 2011
Accepted: 25 May 2011
Published: 25 July 2011
Wegener's granulomatosis is a rare multi-system disease characterized by the classic triad of necrotizing granulomas affecting the upper and lower respiratory tracts, disseminated vasculitis and glomerulonephritis. Oral lesions as a presenting feature are only encountered in 2% of these cases. Hyperplastic gingival lesions or strawberry gingivitis, is a characteristic sign of Wegener's granulomatosis. The latter consists of reddish-purple exophytic gingival swellings with petechial haemorrhages thus resembling strawberries. Recognition of this feature is of utmost importance for timely diagnosis and definitive management of this potentially fatal disease. A case of strawberry gingivitis as the first presenting sign of Wegener's granulomatosis affecting a 50-year-old Malay male is reported here. The differential diagnosis of red lesions that may present in the gingiva is discussed.
KeywordsWegener's granulomatosis strawberry gingivitis oral lesion systemic disease
Wegener's granulomatosis is an uncommon multi-organ disease first categorized as a distinct syndrome by Friedreich Wegener in 1936 [1–3] The hallmarks of this potentially fatal disorder are necrotizing granulomatous inflammation involving the upper and lower respiratory tract, glomerulonephritis, and vasculitis [3–6]. Wegener's granulomatosis may occur as a limited or generalized disease. The limited form of Wegener's granulomatosis runs an indolent course whereas the disseminated disease has a rapid progressive course leading to life-threatening multi-organ failure [2, 7–10]. Of the 2 types of Wegener's granulomatosis, patients with the generalized disease are known to have shorter life expectancy than those presenting with the limited disease. Renal involvement is associated with a more severe outcome for these patients [5, 8]. Wegener's granulomatosis has an insidious onset and usually develops over a period of time with the mean period from onset of symptoms to diagnosis ranging from 4.7 to 15 months [5, 7, 9]. Without treatment it is invariably fatal and most patients do not survive more than a year after diagnosis [11, 12]. Delay in the diagnosis of Wegener's granulomatosis is attributed mostly to the nonspecific presenting signs and symptoms associated with the early phase of the disease [3, 4, 8].
The most characteristic oral lesion is hyperplastic gingivitis, which is typically red to purple with many petechiae (strawberry gingivitis). These lesions may remain localized in the oral cavity for unusually long periods of time before multi-organ involvement occurs [7, 12]. Therefore, timely recognition of this often overlooked oral finding can help to establish an early diagnosis of this disease [4, 8, 11]. Management with appropriate therapy produces a good response in most cases, with only occasional relapses.
A decision was made to perform an incisional biopsy on the gingival growth under local anaesthesia. The specimen obtained was submitted for routine processing followed by staining with haematoxylin-eosin, Periodic acid Schiff, Grocott methenamine silver and Ziehl-neelsen.
The patient was referred to the Medical Department for a systemic work-out. Investigations yielded normal indices for haemoglobin, complete blood count, erythrocyte sedimentation rate and urine analysis. Chest X-rays were also normal. ANCA test was not available.
A decision was taken to treat the patient with prednisolone 60 mg daily and cyclophosphamide 100mg daily.
The American College of Rheumatology (ACR) recommended that the diagnosis of Wegener's granulomatosis can be made if two of the following criteria are fulfilled:1) ulcerative lesions in oral mucosa or nasal bleeding or inflammation, 2) nodules, fixed infiltrates or cavities in chest radiograph, 3) abnormal urinary sediment and 4) granulomatous inflammation on biopsy . In the present case, criteria 1) and 4) were met. Strawberry gingivitis and demonstration of granulomatous inflammation in the gingival biopsy supported the diagnosis of this case as Wegener's granulomatosis. However, there was no history of epistaxis nor pulmonary infiltrates or abnormal urine findings in our patient. Furthermore, his routine blood analysis was also noncontributory.
In summary, a case of strawberry gingivitis presenting as the first sign of Wegener's granulomatosis is described. The clinical lesson from this case report is that early recognition of the characteristic presentation of the gingival lesions of this potentially fatal disorder led to the timely management of the disease.
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