From strawberry gingivitis to palatal perforation: The clinicopathological spectrum of oral mucosal lesions in granulomatosis with polyangiitis.

BACKGROUND: Reports of oral manifestations of granulomatosis with polyangiitis (GPA) usually refer to single-case reports; "strawberry gingivitis" has been increasingly reported. OBJECTIVE: To study the clinicopathological findings of four patients in which the diagnosis of GPA was suspected from the observation of their oral lesions and compare these to existent data. METHODS: Retrospective study of a case series. RESULTS: One patient presented typical "strawberry gingivitis" with localized disease and negative ANCA results. Two patients presented rapidly growing oral ulcers associated with systemic compromise and high ANCA levels. One patient presented with a chronic granulomatous lesion that leaded to palatal perforation. CONCLUSION: Oral manifestations of GPA may vary from rapidly evolving lesions in acutely ill patients to chronic and locally destructive lesions in slowing developing disease. These differences are also evident in the histopathological findings.

Marathon of eponyms: 23 Wegener granulomatosis.

The use of eponyms has long been contentious, but many remain in common use, as discussed elsewhere (Editorial: Oral Diseases. 2009: 15; 185). The use of eponyms in diseases of the head and neck is found mainly in specialties dealing with medically compromised individuals (paediatric dentistry, special care dentistry, oral and maxillofacial medicine, oral and maxillofacial pathology and oral, oral and maxillofacial radiology and maxillofacial surgery) and particularly by hospital-centred practitioners. This series has selected some of the more recognized relevant eponymous conditions and presents them alphabetically. The information is based largely on data available from MEDLINE and a number of internet websites as noted below: the authors would welcome any corrections. This document summarizes data about Wegener granulomatosis.

Aortitis with aneurysm formation as a rare complication of Wegener's granulomatosis.

Wegener's granulomatosis (WG) is a systemic vasculitis of medium-sized and small blood vessels. Aortic involvement in WG is very uncommon. We present a 43-year-old patient with an aortitis with aneurysm formation as a manifestation of WG. The patient was operated on and an aortoiliac Dacron inlay graft was inserted. Postoperatively, he recovered uneventfully. Abdominal pain occurring during a WG flare may result from vasculitis of large abdominal arteries with or without aneurysmatic changes for which surgical treatment and immunosuppressive agents are indicated to prevent a possible rupture.

Strawberry gingivitis as the first presenting sign of Wegener's granulomatosis: report of a case.

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.

Gingival Swelling as the Initial Manifestation of Granulomatosis with Polyangiitis.

We describe a case of granulomatosis with polyangiitis (GPA; formerly named Wegener granulomatosis) that presented initially as florid areas of gingival swelling. The patient also had upper respiratory symptoms that included sinus congestion and cough of recent onset. Clinical-pathologic correlation aided the interpretation of non-specific biopsy findings and immediate referral to an appropriate medical specialist. Treatment was rendered at an early stage of disease with a good response to date. Review of the literature indicates that gingival swelling, often with the characteristic appearance of "strawberry gingivitis" may represent the initial sign of disease in 2% of patients with GPA. Biopsy of gingival lesions often shows a non-specific histologic appearance that should be interpreted in the context of the clinical appearance and pertinent medical history. The clinical investigations that lead to definitive diagnosis and treatment are presented to facilitate the management of this uncommon but potentially fatal condition.

Diagnostic considerations and prosthetic rehabilitation of a cocaine-induced midline destructive lesion: A clinical report.

The intranasal inhalation of cocaine has numerous complications. In addition to its systemic effects, cocaine can cause extensive destruction of the osteocartilaginous midline structures of the palate, nose, and sinuses. Without an accurate social and clinical history, a cocaine-induced midline destructive lesion can cause diagnostic difficulties, because its clinical presentation closely mimics other diseases. This clinical report describes an oronasal defect caused by cocaine use, the diagnostic considerations of these lesions, and prosthetic management of the defect.

An unusual case of Wegener's granulomatosis with tongue involvement.

Wegener's granulomatosis is a type of vasculitis characterized by necrotizing granulomatosis respiratory tract lesions and necrotising glomerulonephritis. Nasal, lung and renal biopsies and positive antineutrophil cytoplasmic antibody (c-ANCA) analysis is helpful in diagnosis. Early diagnosis and treatment is critical in prognosis. A 42 years-old male had dyspnea, coughing, hemoptysis, fatigue, loss of appetite, night sweating and arthralgia. Violaceous palpable, purpuric lesions were detected on the sublingual region of the mouth. On chest X-ray, there was nonhomogeneous infiltration in the parenchyma of both lungs. There was alveolar density involving upper lobes of both lungs detected in thoracal computerized tomography as well as patchy densities on the right lung upper-middle lobes. A nasal mucosa biopsy showed strongly destructive vasculitis. c-ANCA test was positive. We report an unusual case with Wegener's granulomatosis, characterized by a rare presentation of tongue involvement and atypical lung radiology with alveolar opacity.

An Atypical Case of Non-asthmatic Eosinophilic Granulomatosis with Polyangiitis Finally Diagnosed by Tissue Biopsy.

A 78-year-old woman with fever of unknown origin that had persisted for 3 months, systemic edema, and cervical lymphadenopathy was admitted to our hospital. Skin purpura and jaw claudication were subsequently observed. Histopathological examinations of the lymph nodes, skin, and temporal artery revealed findings characteristic of eosinophilic granulomatosis with polyangiitis (EGPA). However, she had no past medical history of asthma with modest eosinophilia. Although EGPA is a systemic vasculitis characterized by asthma and eosinophilia, various limited forms have been described. This was therefore considered to be an atypical form of non-asthmatic EGPA complicating with temporal arteritis (TA) diagnosed by tissue biopsy.

Granulomatosis polyangiitis.

A 22-year-old female patient was admitted to hospital after being referred from the oral medicine clinic where she had been seen for persistent gingivitis and mouth ulcers. She described an insidious history of persistent fevers, dry cough and unexplained weight loss over 4-6 weeks. Imaging showed extensive bilateral pulmonary nodules with mediastinal lymphadenopathy and two lesions in the pancreas. MRI revealed these lesions to be well-defined fluid-filled cysts in the tail of the pancreas, without features of malignancy. Core biopsies taken from her lung nodules demonstrated features of vasculitis with granulomata. This was consistent with her positive immunology for c-antinuclear cytoplasmic antibodies and proteinase-3, which were sent after her fever failed to settle with antibiotic treatment. In keeping with a diagnosis of vasculitis, the patient showed a significant clinical and biochemical response to intravenous methylprednisolone and high-dose daily prednisolone thereafter.

Granulomatosis with polyangiitis: potentially lethal gingival lesions presenting to the dentist.

Gingival pathology is a daily presentation, however a small number of systemic conditions can manifest similar to a common gingival condition and have fatal results. Dentist referred 56-year-old woman to Oral and Maxillofacial Surgery department with a 2-week medical history of gingival bleeding not responding to local measures. Biopsy showed eosinophilic infiltrate and vasculitis, and blood tests showed positive markers including cytoplasmic antineutrophil cytoplasmatic antibodies. Granulomatosis with polyangiitis is a rare disease affecting the respiratory tract, blood vessels and kidneys. Oral lesions are rarely the primary presenting feature. When left untreated, most cases are fatal within a year of diagnosis. The diagnosis can only be made when certain criteria are found, including granular oral lesions exhibiting an eosinophilic inflammatory infiltrate on biopsy. With 5% of cases showing intraoral lesions as the primary feature, it is essential that dentists have the knowledge of this rare disease to refer and not to treat as a common gingival condition.

Oral manifestations of Wegener's granulomatosis: a report of three cases and a literature review.

BACKGROUND: Hyperplastic granular gingivitis or "strawberry gingivitis" is a rare manifestation of Wegener's granulomatosis (WG), but it is nearly pathognomonic for this multisystem autoimmune vasculitis. The dentist may be the first health care professional to see patients with symptoms and findings of this condition. Early diagnosis and treatment is the most important factor in the management of this potentially fatal disease. METHODS: The authors present three case reports that demonstrate the disease spectrum and conducted a literature review focused on current understanding of this disease. RESULTS: The first patient had only the classic gingival manifestations of the disease. The second patient had simultaneous typical gingival lesions, as well as dermatologic findings. The third patient had an atypical oral presentation of aphthous ulcers and erythematous gingiva, as well as respiratory and genital involvement. Reaching a definitive diagnosis sometimes is challenging owing to the subtle onset of the disease and variable clinical and laboratory findings. CONCLUSION AND CLINICAL IMPLICATIONS: Clinicians should be familiar with the broad variety of oral and systemic components of WG, as well as strategies to facilitate prompt disease recognition and to provide continued oral health care to these medically complex patients.

Periodontal diseases.

The periodontal diseases are highly prevalent and can affect up to 90% of the worldwide population. Gingivitis, the mildest form of periodontal disease, is caused by the bacterial biofilm (dental plaque) that accumulates on teeth adjacent to the gingiva (gums). However, gingivitis does not affect the underlying supporting structures of the teeth and is reversible. Periodontitis results in loss of connective tissue and bone support and is a major cause of tooth loss in adults. In addition to pathogenic microorganisms in the biofilm, genetic and environmental factors, especially tobacco use, contribute to the cause of these diseases. Genetic, dermatological, haematological, granulomatous, immunosuppressive, and neoplastic disorders can also have periodontal manifestations. Common forms of periodontal disease have been associated with adverse pregnancy outcomes, cardiovascular disease, stroke, pulmonary disease, and diabetes, but the causal relations have not been established. Prevention and treatment are aimed at controlling the bacterial biofilm and other risk factors, arresting progressive disease, and restoring lost tooth support.

Wegener granulomatosis simulating bacterial endocarditis.

Cardiac involvement in Wegener granulomatosis is uncommon. We report a case of Wegener granulomatosis that presented as culture-negative endocarditis with aortic valvular vegetation. The clinical manifestations included gingival hyperplasia, gangrenous digital infarcts, mononeuritis multiplex, high fever, inflammatory arthritis, pansinusitis, splenic infarct, and aortic valvular vegetation, which underscore the difficulty of distinguishing systemic vasculitis from bacterial endocarditis. Contrary to the common notion that valvular vegetation is invariably associated with bacterial endocarditis, this case proves that such findings can occur in Wegener granulomatosis as well. Clinicians are guided toward early treatment with corticosteroids and cyclophosphamide to prevent fatal complications.