We present the case of a previously healthy 59-year-old man who was under treatment for scleritis and episcleritis when he developed a parotid-gland swelling and pus-producing sinus. On surgical exploration, the features were those of a parotid abscess, but the lesion not only failed to heal post-operatively but increased in size very significantly. There was also severe necrotizing keratitis of the eyes. Due to clinical suspicion and a positive antineutrophil cytoplasmic antibodies test, Wegener’s granulomatosis was diagnosed and the patient successfully treated with cyclophosphamide and steroids. Previously, a number of cases of Wegener’s granulomatosis causing salivary-gland swelling have been reported in the literature; this is the first case in which the disease has masqueraded as a parotid abscess.

We present a case report of an aggressive natural killer T cell lymphoma in a police officer who presented with combined features of orbital cellulitis and mid-facial destruction. However, his initial diagnosis was confused with other disease conditions that had overlapping features. This emphasizes the significance of clinical alertness and adequate tissue sampling; this can have a great impact on early diagnosis and treatment.

Wegener’s granulomatosis is a multisystemic disease characterized by foci of necrotizing vasculitis and granuloma formation. Subglottic stenosis may occur either asa presenting feature or a late-stage manifestation of the disease, but will occur in approximately 10–20 per cent of cases.

We present a series of seven cases of Wegener’s granulomatosis with subglottic stenosis and discuss our management of this condition. Where there is active disease, tracheostomy is the first-line surgical treatment of respiratory obstruction, as an adjunct to full medical therapy.More aggressive or elaborate surgical treatments should be reserved for non-active cases in which patients have not required medical treatment for one year.

A case of Wegener’s granulomatosis (WG) presenting with hearing loss and right facial nerve palsy is reported. The definitive diagnosis was based upon clinical data and serum cANCA and AECA detection. Early assessment of WG prevented surgical facial nerve decompression to treat a chronic otitis media complication. Immunosuppressive therapy with steroids, cyclophosphamide and methotrexate was required for relief of clinical symptoms and cANCA negativity as an expression of disease remission. The effectiveness of co-trimoxazole for preventing relapses of WG is discussed.

Wegener’s granulomatosis is a potentially fatal disease of unknown origin affecting mainly the upper and lower respiratory tracts and kidneys. Prompt recognition of the more unusual presentations of the disease is necessary to ensure early treatment. We present a case of a 46-year-old female with bilateral submandibular and parotid gland enlargement.

Parotid or submandibular salivary gland enlargement is a rare presenting feature of Wegener’s granulomatosis. Common to 80 per cent of these cases is nasal involvement, while ear pathology or lung lesions may occur in the remaining cases. The diagnosis is both clinical and pathological, biopsy of suspicious tissue, serum c-ANCA levels and a chest X-ray are valuable investigations. Treatment with immunosuppressive therapy is essential, and usually ensures a long-term remission.

A Th1/Th2 cytokine imbalance with a predominance of Th1 cytokines has been suggested to be of pathogenic importance in Wegener’s granulomatosis. To evaluate the role of Th1/Th2 cytokines in Wegener’s granulomatosis, the subsets of Th1, Th2, Tc1 and Tc2 cells from patients with active Wegener’s granulomatosis were examined by intracellular cytokine flow cytometry. The population of Tc1 cells (72.0 ± 14.4 per cent) in Wegener’s granulomatosis was significantly increased compared with Tc1 cells (37.3 ± 14.6 per cent) in control (p<0.05). Th1, Th2 and Tc2 cells in Wegener’s granulomatosis were not significantly increased compared with the control cells. These results indicate that the predominance of Tc1 cells might contribute to the mechanism of the pathogenesis of Wegener’s granulomatosis.

This is a retrospective review of 74 consecutive patients who presented with a nasal septal perforation or ulcer. The aim of this study was to evaluate the contribution of the investigations used to identify the pathology underlying their septal perforation or ulcer. In the majority of cases there was an anterior (85 per cent) smooth, well-circumscribed septal lesion with an occasional bleeding point. Seventy-seven per cent were less than two cm in diameter. The primary presenting symptom was intermittent epistaxis, with the exception of patients with granulomatous disease, who presented more frequently with nasal obstruction. Thirty-five patients (47 per cent) were idiopathic, 29 (39 per cent) traumatic, eight (11 per cent) inflammatory and two (three per cent) infectious. The nasal septum was biopsied in 71 cases, and histology showed non-specific or non-diagnostic findings in 39 (55 per cent), chronic inflammation in 12 (17 per cent), acute inflammation in six (eight per cent), acute to chronic inflammation in four (six per cent) and histological changes consistent with trauma in nine (13 per cent). The anti-neutrophil cytoplasmic antibody test (ANCA) successfully identified six patients with Wegener's granulomatosis, and the angiotensin converting enzyme test (ACE) supported a clinical diagnosis of sarcoidosis in one patient. The remaining investigation methods (CRP, ESR, chest radiograph, biochemistry and coagulation screen) did not contribute to the diagnosis. In this study, the appearance of a nasal septal perforation or ulcer or its location did not predict the clinical diagnosis. A nasal septal bopsy did not significantly contribute toward the clinical diagnosis. On the basis of these findings, a biopsy of a septal perforation or ulcer is unlikely to contribute to the management of patients unless the supportive tests of ANCA or ACE are abnormal or unless malignancy is suspected. This conclusion is restricted to the management of septal perforations as we have found nasal biopsies of polyps of other lesions to be of value.

Wegener’s granulomatosis is a systemic disease characterized by a granulomatous lesion that can affect any organ throughout the body.

This case-report illustrates the problem posed by a patient presenting with bilateral serous otitis media with marked sensorineural hearing loss, facial nerve paralysis and an atypical serological picture. The importance of early diagnosis and the protocol for the management of a patient with an uncertain diagnosis is discussed. Due to atypical presentations, only a high index of suspicion will ensure an early diagnosis.