Granulomatous cheilitis of Miescher: a rare entity.

BACKGROUND: Granulomatous cheilitis (GC) is a rare entity of unknown etiology. It is a chronic inflammatory disorder with a predilection for young females. It is characterized by asymptomatic unrelenting swelling of lips. It is a monosymptomatic form or an incomplete variant of Melkersson-Rosenthal syndrome (a triad of recurrent orofacial swelling, relapsing facial paralysis, and fissuring of the tongue). CASE PRESENTATION: We herewith report a case of a 27-year-old female, presenting with persistent upper lip swelling, for 3 months. Biopsy from the lesion revealed features of granulomatous cheilitis. CONCLUSION: GC should be considered in the differential diagnosis of unrelenting swelling in the lip. Spontaneous remission is rare, and recurrences are common. Corticosteroids used for treatment provide temporary improvement.

Orofacial granulomatosis: Clinical and therapeutic features in an Italian cohort and review of the literature.

BACKGROUND: Orofacial granulomatosis (OFG) is characterized by granulomatous inflammation of the soft tissues of maxillofacial region. We explored OFG patients from 10 different Italian centers and summarized the most recent literature data. METHODS: A review of patients with OFG was carried out. An extensive online literature search was performed to identify studies reporting diagnosis and management of OFG. RESULTS: Thirty-nine patients were recruited between January 2018 and February 2020. Most of them (97.4%) displayed involvement of the lips, and 28.2% suffered from Melkersson-Rosenthal syndrome. Two patients received diagnosis of CD and one patient of sarcoidosis, suggesting secondary OFG. Oral aphthosis and cervical lymphadenopathy were also described. The mean diagnostic delay was 3.4 years. Histological evaluation was performed in 34/39 patients (87.2%); non-caseating granulomas were found in 73.5% of them. Neurological symptoms (28.2%), gastrointestinal symptoms in absence of overt inflammatory bowel disease (IBD) (20.5%), and atopy (35.9%) were also identified. Therapeutic approaches varied among the centers. Steroids (51.3%) were used with good or partial results. Anti-TNF-α and anti-IgE monoclonal antibodies were used in 6 (15.4%) and 1 (2.6%) patients, respectively, with variable results. Surgery was the choice for 2 patients with good response. CONCLUSIONS: OFG is a rare and neglected disease showing multiple clinical phenotypes. While early diagnosis is crucial, management is difficult and highly dependent on the expertise of clinicians due to the lack of international guidelines. There is a need to establish registry databases and address challenges of long-term management.

A case of orofacial granulomatosis evolving into Melkersson Rosenthal syndrome in a child treated with a steroid free regimen of Clofazimine and Minocycline.

Orofacial granulomatosis (OFG) is a rare disorder with varied etiological, immunological and infectious mechanisms implicated and is believed to be a umbrella term which includes Melkersson Rosethal syndrome (MRS). We describe a 17 year old female who was diagnosed with OFG and was successfully treated with a combination of minocycline and clofazimine without oral steroids with significant improvement within 1 month of therapy.

Clinical Profile of Melkersson-Rosenthal Syndrome/Orofacial Granulomatosis: A Review of 51 Patients.

BACKGROUND: Melkersson-Rosenthal syndrome (MRS) is a rare disease characterized by the triad of granulomatous cheilitis, fissured tongue, and facial paralysis. Publications concerning large series are rare in the literature. OBJECTIVES: To describe the clinical and histopathological characteristics of patients with complete and oligosymptomatic forms of MRS. METHODS: A retrospective records review was performed for the diagnoses of Melkersson-Rosenthal syndrome, granulomatous cheilitis, and orofacial granulomatosis at oral Diseases Clinic of the Department of Dermatology, University of São Paulo, Brazil (2003, 2017). RESULTS: A total of 51 patients were included, mean age at presentation 35.69 years. Four patients were younger than 18 years. The complete triad of was observed in 10 patients. The rare findings of granulomatous blepharitis, gingivitis and palatitis are presented. Comorbidities included Crohn's disease (5 patients), migraine headaches (1 patient) and convulsions (2 patients). Granulomatous inflammatory infiltrate was detected in 31 biopsies. Medical therapies included included oral and intralesional steroids, thalidomide, dapsone, azathioprine, tetracycline, methotrexate, and surgery, with variable responses. CONCLUSIONS: Our report meant to draw attention to the clinical spectrum of this rare disorder, mainly to oligosymptomatic forms and rarer presentations.

An uncommon case of lip swelling: Granulomatous cheilitis associated with Crohn's disease.

Unbeknownst to most otolaryngologists, there is quite a range of oral manifestations which commonly manifest in the context of inflammatory bowel disease. As providers who will encounter such patients in consultation it is beneficial to be aware of that association. Lip swelling (granulomatous cheilitis) is just one such presentation, which is often otherwise mistaken for angioneurotic edema and can lead to unwarranted testing and misdirected treatment. We present such a case to highlight the educational value of this patient encounter.

Research progress on Melkersson-Rosenthal syndrome.

Melkersson-Rosenthal syndrome (MRS) is a rare neuro-muco-cutaneous syndrome, which is characterized by recurrent orofacial swelling, recurrent facial paralysis and fissured tongue. It has a high prevalence in young adults. Up to now, the etiology of MRS is still not clear, it may related to infection, immune deficiency and hereditary factors. The pharmacological therapy and surgery are the main treatment. Corticosteroids seems to be the drug of choice for MRS patient, but the specific dosage and therapeutic effect have not yet been determined. Surgeries of lips provide excellent results in persistentlip edema MRS cases. This article reviews the research progress on MRS, focusing on its epidemiology, etiology, histopathological characteristics, clinical manifestations, classification, diagnostic criteria, differential diagnosis and treatment, to provide information for its early diagnosis and appropriate treatment.

Morbihan disease: a case report and differentiation from Melkersson-Rosenthal syndrome.

We present a 32-year old woman with a 9-year history of upper facial swelling. A workup by the ophthalmology department led to the diagnosis of Melkersson-Rosenthal syndrome. Re-evaluation in our dermatology clinic confirmed a diagnosis of Morbihan disease. Herein, we review case reports and case series of upper facial swelling in the dermatologic and ophthalmologic literature. Although the two entities share histopathological changes, they tend to have different clinical presentations. Melkersson-Rosenthal syndrome appears to be more likely diagnosed in the ophthalmologic literature when the clinical presentation and histopathology may be more consistent with Morbihan disease. In a patient with upper facial swelling, an absence of orolabial swelling, and lack of facial neuropathy, we argue for a diagnosis of Morbihan disease over Melkersson-Rosenthal syndrome, especially if the patient has a history of rosacea.

Resident Rounds: Part III--Case Report: Crohn's Disease Presenting as Granulomatous Cheilitis.

Cutaneous Crohn's is a rare extra-intestinal manifestation of inflammatory bowel disease seen in a select group of patients, in which cutaneous lesions similar to those of the intestinal illness appear distant from the gastrointestinal tract. Oral findings may be found in up to 60% of patients with extra-intestinal Crohn's and may appear as the initial symptom underlying disease. We present a case of a 17-year-old male presenting with granulomatous chelitis of the lower lip who was unaware of the underlying diagnosis of Crohn's disease (CD).

Periocular intralymphatic histiocytosis or localized Melkersson-Rosenthal syndrome?

We describe three cases of periocular edema with histopathologic features of intralymphatic histiocytosis without extravascular granulomas. All were elderly males with no other significant medical problems. Previous reports of periocular Melkersson-Rosenthal syndrome are identical clinically, and some reports show illustrations of intralymphatic histiocytosis histopathologically, in addition to other features typical of the syndrome. Given the lack of associated diseases or other features of the Melkersson-Rosenthal triad, some of these cases may be better defined as periocular intralymphatic histiocytosis.