To identify patients evaluated in an outpatient setting at our institution with a presentation of recurrent orofacial swelling and to review the spectrum of causes to outline a diagnostic approach.

A retrospective study of 104 patients with more than 1 episode of orofacial swelling lasting for more than 5 days identified through a keyword search of the electronic health record from January 2, 2000, through July 5, 2011.

Patients were categorized according to final cause of orofacial swelling: idiopathic orofacial granulomatosis, solid facial edema due to rosacea and acne vulgaris, Crohn disease, contact dermatitis, sarcoidosis, exfoliative cheilitis, lichen planus, actinic cheilitis, cheilitis glandularis, lymphedema, miscellaneous, and multifactorial. Granulomatous inflammation was noted on biopsy in 40 of 85 patients (47%). Oral involvement was associated with Crohn disease (P<.001), and facial and periorbital swelling was associated with solid facial edema in the setting of rosacea and acne vulgaris (P<.001).

The broad range of diagnoses responsible for recurrent orofacial swelling underscores the diagnostic challenge and importance of a thorough multidisciplinary evaluation to identify underlying causes.

Orofacial granulomatosis (OFG) is an uncommon chronic inflammatory disorder of the orofacial region. It is characterized by subepithelial noncaseating granulomas and has a spectrum of possible clinical manifestations ranging from subtle oral mucosal swelling to permanent disfiguring fibrous swelling of the lips and face. Etiopathogenesis is unknown. A range of systemic granulomatous disorders, including Crohn disease and sarcoidosis, may cause orofacial manifestations that cannot be distinguished from those of OFG. Treatment of OFG has proven difficult and unsatisfactory, with no single therapeutic model showing consistent efficacy in reducing orofacial swelling and mucosal inflammation.

We report three cases of orofacial granulomatosis (OFG) to illustrate the spectrum of this disease, and to discuss the appropriate management steps, consider its overlap with Crohn's disease (CD) and raise its awareness among paediatric dermatologists. The term 'orofacial granulomatosis' was first used in 1985 to describe granulomas in the orofacial region in the absence of any recognized systemic condition. It is uncommon but becoming increasingly recognized in children. The clinical features of the disease may vary greatly, and often present with subtle changes that can be missed. There is a debate about whether OFG exists as a separate condition or whether it is an oral feature of CD, as some patients go on to develop CD several years later. Identifying those most at risk is important, as ongoing investigations may be necessary. The three cases presented in this series illustrate the range of disease signs and symptoms, and the investigations required.

Orofacial granulomatosis (OFG) is a rare clinical entity with orofacial swelling in association with oral lesions such as mucosal oedema, ulcerations and mucosal tags. The aim of this prospective study was to evaluate the connection between OFG in childhood and systemic disease.

During a 3-year period, eight children (9-16 years old) who had been referred to the clinic of oral medicine were diagnosed solely with OFG. Thus, none of them had any known systemic disease or gastrointestinal symptoms at the time of referral. The children were then medically examined and followed up for 6-8 years at the department of paediatrics for systemic disease with specific emphasis on inflammatory disorders elsewhere in the gastrointestinal tract.

During follow-up, four patients were diagnosed with Crohn's disease (CD). Further, one girl was found to have a food allergy-induced OFG, with delayed-type hypersensitivity to oats. One boy developed both diabetes and celiac disease during the follow-up. Only two patients had no diagnosis of systemic disease at the end of the observation period.

OFG in childhood seems to be frequently related to systemic disease, and children with OFG should be referred to a paediatrician for examination and follow-up.

Orofacial granulomatosis (OFG) is the presence of persistent enlargement of the soft tissues of the oral and maxillofacial region, characterized by non-caseating granulomatous inflammation in the absence of diagnosable systemic Crohn's disease (CD) or sarcoidosis. Over 20 years have passed since OFG was first described and an extensive review of the literature reveals that there is no consensus whether OFG is a distinct clinical disorder or an initial presentation of CD or sarcoidosis. Furthermore, the precise cause of OFG is still unknown although several theories have been suggested including infection, genetic predisposition and allergy. The clinical outcome of OFG patients continues to be unpredictable. Current therapies remain unsatisfactory. Regular clinical review is indicated to identify the development of gastrointestinal or systemic involvement. The aim of this review was to analyse the developments in our understanding of the aetiology, pathogenesis and treatment protocols, with particular emphasis on management and outcomes of OFG since this entity was first described in 1985.

We aimed to attract our college's attention to the Melkersson-Rosenthal syndrome (MRS), which has been an infrequently encountered subject in otolaryngology journals during the last 10 years.

A retrospective review of the last 10 years' patient database was performed to find patients with MRS. The medical files, treatment charts, and radiological and histopathological records of these patients were reviewed.

The study group consisted of 3 MRS patients who had been misdiagnosed for 9, 10, and 16 years. Two of them have had the symptoms since adolescence. All of them presented orofacial edema and fissured tongue, whereas first two also had recurrent facial paralysis. Characteristic histopathological features were noted in 1 patient. Electromyography (EMG) was done in 1 patient who underwent facial decompressiom. All patients responded to either systemic or intralesional corticosteroid treatment.

In the daily practice of an otolaryngologist, it is not usual to diagnose a patient as having MRS. We consider that this is partly because of misdiagnosis. We therefore believe that this study will supply an additional aspect to otolaryngologists, in the scope of recurrent facial paralysis and orofacial edema in both children and adults.

Melkersson Rosenthal Syndrome (MRS) is a rare disorder characterized by relapsing facial paralysis, persistent or recurrent orofacial edema, and lingua plicata. It may cause difficult airway, drug allergy, and angioedema. In our anesthetic management of two patients with MRS, preanesthetic immunological blood examination and skin tests for hypersensitivity to anesthetic drugs were applied. Because the principal goal is to avoid all factors that may stimulate, an allergic reaction, anesthetic drugs known to trigger urticaria were avoided. Body and operating room temperatures, changes of which may trigger allergic reactions, were kept constant during the perioperative period. Emergency precautions were taken for probable angioedema. MRS is a rare syndrome, and if its manifestations are misunderstood as simple facial paralysis, it may be overlooked by anesthesiologists. Anesthesiologists must be careful of several problems in patients with MRS.

Orofacial granulomatosis (OFG) is considered as an uncommon disease and nomenclature of the disease was subjected to debate for a long time. Although various aetiological agents such as food substances, food additives, dental materials and various microbiological agents have been implicated in the disease process its precise pathogenesis is yet to be elucidated. Delayed type of hypersensitivity reaction appears to play a significant role, although the exact antigen inducing the immunological reaction varies in individual patients. However, evidence for the role of genetic predisposition to the disease is sparse. The underlying immunological mechanism appears to show some similarities between OFG and Crohn's disease, emphasizing the need for more comparative studies of the two entities. Therefore, we propose the term idiopathic OFG as a better term for those cases restricted to oral region without any identifiable known granulomatous disease and the diagnosis should not be changed until the patient develops systemic manifestations of a specific granulomatous condition. This review attempts to discuss the role of different aetiological agents and certain aspects of pathogenesis of OFG.

Melkersson-Rosenthal syndrome is an unusual cause of facial swelling that can be confused with angioedema.

To describe a young woman with facial swelling initially considered to be angioedema.

A biopsy specimen of the eyelid demonstrated findings consistent with Melkersson-Rosenthal syndrome.

After reviewing the differential diagnosis of pseudoangioedema, a presumptive diagnosis of Melkersson-Rosenthal syndrome was made. The patient was successfully treated with infliximab for Melkersson-Rosenthal syndrome. Owing to medication adverse effects, infliximab treatment was discontinued. Treatment was then continued with adalimumab, with good effect and without adverse events.

We report the case of a patient with Melkersson-Rosenthal syndrome presenting as angioedema. Furthermore, we report the first successful treatment of Melkersson-Rosenthal syndrome with adalimumab.

Orofacial granulomatosis, an entity with characteristic clinicopathologic features, is thought to be a reactive process. The authors describe orofacial granulomatosis associated with contact allergy to gold in dental crowns in one patient and a possible allergic contact reaction to mercury from dental fillings in another one. Thus allergic contact dermatitis to the metals gold and mercury should be considered as a possible etiologic agent of orofacial granulomatosis.

A 61-year-old woman presented with a 2-year history of an abnormal erythematous swelling on the upper lip and cheek. Upon examination there were no other physical findings. Histological examination found discreet sarcoidal granulomas in the lower dermis. Routine laboratory studies, chest radiographs and pulmonary functions were all normal. Clinical presentation and histological findings were, therefore, compatible with the diagnosis of orofacial granulomatosis (OFG). The patient was patch tested with an extended standard series that included metal-salt, dental prosthesis, bakery and corticosteroids series. The patch test was positive (score ++) after 48 and 72 h for mercury in the metal-salt and dental prosthesis series. During the past decade the patient had received amalgam fillings of several dental cavities, including one adjacent to the swollen cheek. The unilateral localization of the soft tissue swelling adjacent to the amalgam tooth fillings, along with the positive patch test for mercury, raised the possibility that the OFG was part of a delayed hypersensitive reaction to the fillings. The patient therefore underwent a total amalgam replacement procedure; complete disappearance of the swelling overlying the right cheek was observed within 7 weeks and the swelling of the upper lip subsided completely within 6 months. We propose that mercury in amalgam tooth fillings is another cause of OFG and suggest appropriate patch testing in patients who do not have an apparent cause of OFG.

Melkersson-Rosenthal syndrome (MRS) is a rare disorder of unknown etiology characterized by a triad of symptoms: recurrent orofacial swelling, relapsing facial palsy. and a fissured tongue. A differential diagnosis must be made with other granulomatous diseases, such as sarcoidosis and oral Crohn disease; however, the histologic findings of noncaseating, sarcoidal granulomas support the diagnosis of MRS.

Many therapeutic modalities have been described for this disease. In this case report, we present a patient with MRS that was treated with thalidomide because of the identification of tumor necrosis factor a in the lesion by immunohistochemical analysis. This is the first reported detection of tumor necrosis factor a in an MRS lesion, as well the first reported use of thalidomide to treat this clinical condition.