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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 32  |  Issue : 3  |  Page : 293-296

Insidious fatal perforation of palate: A diagnostic challenge


Department of Oral Medicine and Radiology, H. P. Government Dental College and Hospital, Shimla, Himachal Pradesh, India

Date of Submission27-Mar-2020
Date of Decision06-Jun-2020
Date of Acceptance12-Jun-2020
Date of Web Publication29-Sep-2020

Correspondence Address:
Dr. Akshay Sujith
Junior Resident, Department of Oral Medicine and Radiology, H. P Government Dental College and Hospital, Shimla, Himachal Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.jiaomr_52_20

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   Abstract 


Granulomatosis with polyangiitis (GPA) is considered as a systemic triad of necrotizing granulomatous vasculitis of the upper and lower respiratory tracts (pulmonary vasculitis) and a rapidly progressive glomerulonephritis. Few cases of a superficial form with only oral involvement are reported. The purpose of this article is to describe the steps in reaching a conclusive diagnosis in lesions involving the palate. Since in most cases the histopathological report will turn inconclusive, proper clinical evaluation and definitive diagnostic test like the estimation of cytoplasmic-antineutrophilic cytoplasmic antibody will be mandatory for obtaining a conclusive result.

Keywords: Antineutrophilic cytoplasmic antibodies, granulomatosis polyangiitis, perforation of palate, Wegener's granulomatosis


How to cite this article:
Prasad R G, Sujith A, Sharma N, Kumar B. Insidious fatal perforation of palate: A diagnostic challenge. J Indian Acad Oral Med Radiol 2020;32:293-6

How to cite this URL:
Prasad R G, Sujith A, Sharma N, Kumar B. Insidious fatal perforation of palate: A diagnostic challenge. J Indian Acad Oral Med Radiol [serial online] 2020 [cited 2020 Dec 3];32:293-6. Available from: https://www.jiaomr.in/text.asp?2020/32/3/293/296586




   Introduction Top


Granulomatosis with polyangiitis (GPA) is a potentially lethal systemic disorder that is characterized by necrotizing vasculitis of small arteries and veins.[1] The disease was first described as a form of polyarteritis nodosa by Klinger, unique nature of the disease was described by Wegener, and the term GPA was coined by Godman and Churg.

The incidence of GPA is estimated to be 8–10 cases per million affecting both men and women equally, with men more likely to have a severe form of the disease. The peak incidence is seen at 64–75 years of age. Studies state that in 8%–15% cases GPA can occur in individuals aging 19 years or younger.[2]

Three forms of GPA formerly called as Wegener's granulomatosis (WG) have been reported in literature. The primary or classic triad includes necrotizing granulomatous vasculitis, pulmonary vasculitis, and glomerulonephritis, with poor prognosis. The limited form having only respiratory involvement has a better prognosis. In some cases, it may also present with initial oral lesions and later showing multisystem involvement in a few weeks or months.[3]

The etiology of GPA is unknown; genetic susceptibility coupled with the environmental triggering factor leading to vasculitis has been implicated in the initiation of the disease. The strongest genetic correlation found associated with the disease refers to HLA-DRB1*1501 apart from which α1-antitrypsin (α1AT) deficiency was also implicated. Association of disease with infections, especially during the winter months is a debatable predisposition as per the literature. GPA is associated with circulating antibodies against cytoplasmic components of neutrophils (antineutrophilic cytoplasmic antibodies, ANCA).[4]


   Case Report Top


A 17-year-old male patient who was primarily admitted in the Department of ENT reported to our OPD for dental evaluation. The chief complaint of the patient was painful ulcer in the palate for the past 1 month and subsequent perforation of the palate since 5 days [Figure 1]. The onset of ulcer was insidious which progressively lead to the perforation of the palate. There was a history of dysarthria, dysphonia, dysphagia, and regurgitation of food and water from the nose. The patient complained about intermittent fever, nasal congestion, dry cough, respiratory distress, and weight loss. There was no history of trauma, difficulty in micturition, or any other systemic illness or substance abuse.
Figure 1: Perforation in palate

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On examination, there was a diffuse erosive patch seen on the anterolateral aspects of the hard palate. The lesion had a necrotic slough in the mid-portion of the palate and a perforation of approximately 1.5 cm diameter left lateral to the mid palatal region [Figure 1]. The lesion was tender with no induration, bony crepitus, and no history of any sensory deficit. Regional lymph nodes were not palpable.

A provisional diagnosis of palatal perforation secondary to a granulomatous disease was made and under differential diagnosis, malignant minor salivary gland tumor, perforation secondary to bacterial or fungal infection, GPA, mucormycosis, necrotizing sialometaplasia, and lethal midline granuloma were considered. A radiographic investigation by maxillary occlusal topographic view [Figure 2] revealed an ill-defined ovoid radiolucency in the palate of size approximately 1.5 cm in diameter positioned slightly left lateral to the mid palatal suture. Contrast-enhanced computed tomography (CECT) neck revealed a perforation in the hard palate measuring 17 × 11 mm. There was a deviation of nasal septum toward the right side with soft tissue density suggestive of mucosal hypertrophy involving bilateral nasal cavities. There was the presence of soft tissue densities in the bilateral maxillary, ethmoid, and sphenoid sinuses. Scanned bilateral lungs showed multiple ground-glass opacities and soft tissue nodules, largest measuring 4 mm in the left lung [Figure 3]. The radiographic report was more favorable to GPA, lethal midline granuloma, and necrotizing sialometaplasia.
Figure 2: Maxillary occlusal topographic view showing ill-defined radiolucency suggestive of perforation in the hard palate

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Figure 3: Axial (a,b), coronal, (c) and sagittal (d) section of CECT neck, contrast 40 mL at 2.5 mL/s showing perforation in the palate and soft tissue density in the bilateral maxillary, ethmoid, and sphenoid sinuses. (e) Scanned image of lung showing soft tissue nodule

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Complete blood count and glomerular filtration rate were within normal limits. The random plasma glucose level was 113 mg/dL. Cartridge-based nucleic acid amplification test (CBNAAT) for tuberculosis and HIV test was negative. Incisional biopsy was performed from the advancing edge of the ulcer collaboratively with the department of ENT and soft tissue bit of size 0.5 × 0.4 × 0.3 cm was sent for the histopathological examination, which revealed focally ulcerated stratified squamous epithelium showing round hyperchromatic cells, epithelial artifacts, sheets of neutrophils, histiocytes, and mononuclear cells. No fungal filaments or granuloma was seen [Figure 4]. Cytoplasmic-antineutrophilic cytoplasmic antibody (C-ANCA) under immunofluorescence showed an equivocal titer of 1:20. All other serological tests proved negative. The culture report obtained from the swab was nonspecific.
Figure 4: Inflammatory cell infiltrates

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The patient was administered with empirical antibiotic therapy, inj. amoxicillin and clavulanic acid combination 1.2 g BD and inj. lornoxicam 8 mg BD for 5 days, and prednisolone 40 mg tablets was given orally for 5 days along with 0.2% chlorhexidine. A palatal obturator with proper spacer was fabricated once the lesion had become less tender. The obturator was given only for feeding purpose since the nutritional status of the patient was deteriorating considerably [Figure 5]. After consultation with an otolaryngologist, a pulmonologist, and a nephrologist, a collective decision was taken to start inj. cyclophosphamide 600 mg IV every 21 days. Unfortunately, the patient died due to respiratory distress and hematemesis during the course of treatment.
Figure 5: (a and b) Obturator fabricated for the patient

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   Discussion Top


In 1990, the American College of Rheumatology created guidelines for diagnostic criteria for WG, which included “nasal or oral inflammation,” with the development of painful or painless ulcers or purulent or bloody nasal discharge; “abnormal chest radiograph” showing nodules, fixed infiltrates, or cavities; “urinary sediment” with microhematuria or red cell casts; and “granulomatous inflammation on biopsy” of artery or arteriolar tissue.[5] A patient can be diagnosed with WG if at least two of these criteria are met, in addition to the C-ANCA analysis, the positive cut off is 1:20.[6]

GPA is a multisystem disease with upper respiratory tract involvement seen in 85% of the population, nodular infiltration of lung in 55%–90%, glomerulonephritis in 70%–85%, nasopharynx in 60%–80%, and hearing loss in 15%–25% of population. Chronic sinusitis, epistaxis, otitis media are the predominant clinical features, while deviation of nasal septum, saddle nose, nasal septal perforation, and sore throat are other common clinical features. Intraoral palate and gingiva (strawberry gingivitis) are major sites. Other system involvement includes cutaneous lesion with nonspecific maculopapular rashes; cardiac and gastrointestinal tract lesions are rarely seen.[6] A brief literature review is presented in [Table 1].
Table 1: Brief Literature Review

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The incidence of GPA is less in young age, and also this site of involvement is rarely mentioned in the literature.[3],[4],[5],[6] Our diagnosis was supported by the presence of oronasal inflammation, abnormal nodules seen in chest scan, and also confirmed by the positive titer of C-ANCA. Tuberculosis was ruled out from a negative report of CBNAAT. Histopathological examination helped to rule out the possibility of malignancy and fungal infection; negative serological markers for T cells and NK cells eliminated the possibility of central lethal granuloma.

Mainstream treatment modalities are shown in [Table 2]. Other chemotherapeutic agents include azathioprine, trimethoprim, leflunomide, etc., are used majorly for the maintenance of remission. However, several combination therapy and pulsed dosage have been considered for the treatment. Administration of drugs must be carefully monitored since most of the drug possesses several side effects. Nasal reconstruction and speech therapy should be considered in the later phase of the treatment.[13]
Table 2: A brief description of mainstream treatment modalities

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   Conclusion Top


Oral physician should be familiar with the evaluation and diagnosis of lesions involving the palate since few of them can end up with lethal complications. GPA can lead to multiple systemic complications. Prompt referral to different disciplinarians should be done in order to avoid morbidity and mortality.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the guardian has given their consent for patient images and other clinical information to be reported in the journal. The guardian understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Glick M. Burket's Oral Medicine PMPH-USA 12th edition, Chap: 20; 2015. p. 515.  Back to cited text no. 1
    
2.
Kubaisi B, Samra K, Foster CS. Granulomatosis with polyangiitis (Wegener's disease): An updated review of ocular disease manifestations. Intractable Rare Dis Res 2016;5:61-9.  Back to cited text no. 2
    
3.
Rahilly G, Rahilly M. A case of palatal Wegener's granulomatosis. Oral Diseases 2008;6:259-61.  Back to cited text no. 3
    
4.
Gibelin A, Maldini C, Mahr A. Epidemiology and etiology of Wegener granulomatosis, microscopic polyangiitis, churg-strauss syndrome and goodpasture syndrome: Vasculitides with frequent lung involvement. Semin Respir Crit Care Med 2011;32:264-73.  Back to cited text no. 4
    
5.
Frasier L, Hoag AK. Differential diagnosis of Wegener's granulomatosis from other small vessel vasculitides. Lab Med 2007;38:437-9.  Back to cited text no. 5
    
6.
Lynch PJ, Derhovanessian A, Tazelaar H, Belperio JA. Granulomatosis with polyangiitis (Wegener's granulomatosis): Evolving concepts in treatment. Semin Respir Crit Care Med 2018;39:434-58.  Back to cited text no. 6
    
7.
Kosałka J, Bazan-Socha S, Zugaj A, Ignacak M, Żuk J, Sokołowska B, et al. Granulomatosis with polyangiitis (Wegener's granulomatosis) with hard palate and bronchial perforations treated with rituximab — A case report. Pneumonol Alergol Pol 2014;82:454-7.  Back to cited text no. 7
    
8.
Szczeklik K, Włudarczyk A, Wawrzycka-Adamczyk K, Górka J, Fuks-Kulska M, Darczuk D, et al. Oral manifestations of granulomatosis with polyangiitis-Clinical and radiological assessment. J Dent Sci 2019;14:54-60.  Back to cited text no. 8
    
9.
Langford CA. Update on the treatment of granulomatosis with polyangiitis (Wegener's). Curr Treat Options Cardiovasc Med 2012;14:164-76.  Back to cited text no. 9
    
10.
Langford CA, Talar-Williams C. Staged approach to the treatment of Wegener's granulomatosis: Induction of remission with glucocorticoids and daily cyclophosphamide switching to methotrexate for remission maintenance. Arthritis Rheum1999;42:2666-73.  Back to cited text no. 10
    
11.
Langford CA, Talar-Williams C, Barron KS, Sneller CM. Use of a cyclophosphamide-induction methotrexate maintenance regimen for the treatment of Wegener's granulomatosis: Extended follow-up and rate of relapse. Am J Med 2003;114:463-9.  Back to cited text no. 11
    
12.
Keogh KA, Ytterberg SR, Fervenza FC. Rituximab for refractory Wegener's granulomatosis: Report of a prospective, open-label pilot trial. Am J Respir Crit Care Med 2006;173:180-7.  Back to cited text no. 12
    
13.
Regan MJ, Hellmann DB, Stone JH. Treatment of Wegener's granulomatosis. Rheum Dis Clin North Am 2001;27:863-86.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
 
 
    Tables

  [Table 1], [Table 2]



 

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