Parotid Mass and Orofacial Granulomatosis—A Diagnostic Dilemma with Review of Literature

Introduction: Parotid mass and facial nerve palsy are common clinical presentations in hospital and clinic-based practice. But when presented together they usually point to the possibility of malignancy or inflammatory pathogenesis. Melkersson–Rosenthal Syndrome is one of the diseases in the spectrum of orofacial granulomatosis characterized by bilateral alternate facial nerve palsy, swelling of lips and fissured tongue. We present a study of two cases where the spectrum of disease consisted of parotid mass, alternating facial nerve palsy, characteristic features of Melkersson–Rosenthal syndrome, ultimately leading to the diagnosis of Wegener's granulomatosis.

Study Design: Retrospective case study of two subjects, in the span of 9 years, presenting with unilateral parotid mass, lower motor neuron-type facial palsy, characteristic facial changes and clinicopathological features of Wegener's granulomatosis.

Materials and Methods: Data related to clinical features, pathological and radiological parameters were evaluated in each patient for 15.5 months (range, 7–24 months). Experience from the first case helped us to initiate necessary management plan tailored to second patient. Modified American College of Rheumatology criteria for the classification of Wegener's granulomatosis was followed for assessment.

Results: The primary complaint of both patients was recently developed facial palsy and mild facial puffiness with preexisting parotid mass and otitis media with effusion (OME). Facial palsy improved with parotidectomy and steroid therapy, though OME persisted. Histopathology showed noncaseating granulomatous disease of parotid. Blood levels of c-ANCA and p-ANCA were negative initially, though in the first case there was rising titer of c-ANCA in later stage of disease. After an apparently smooth postoperative recovery there was relapse of facial palsy on opposite side with swollen lips, face and constitutional symptoms. This time lip biopsy showed chronic inflammatory cell predominance with granuloma formation (case 2). The first patient had weight loss, arthralgia, hemoptysis with multiple lung nodules on radiography and was treated with anti-tubercular drugs. She had little improvement and underwent CT guided FNAC from mediastinal mass and pleural biopsy that again showed noncaseating granulomatous lesion, along with high normal level of c-ANCA. Unfortunately, she succumbed to massive hemoptysis within 3 days of starting cytotoxic therapy. In the second case, patient improved dramatically with steroid and methotrexate therapy with only two relapses of facial palsy with lip swelling and epistaxis in last seven years. Renal function and urinalysis were absolutely normal in both cases.

Conclusion: Wegener's granulomatosis can present in generalized or limited form with or without systemic involvement in later period of disease spectrum. Parotid mass is its rare presentation and association with facial palsy and features of orofacial granulomatosis (Melkersson–Rosenthal syndrome) is even rarer. High degree of clinical suspicion and meticulous follow up is needed to establish the diagnosis. Tuberculosis is a common compounding factor in countries like India and must be ruled out to prevent delay in treatment and mortality. Dependence upon antineutrophilic cytoplasmic antibody should be weighed judiciously against clinical symptoms and biopsy reports.