Cheilitis granulomatosa and Melkersson-Rosenthal syndrome: evaluation of gastrointestinal involvement and therapeutic regimens in a series of 14 patients.
نویسندگان
چکیده
BACKGROUND Cheilitis granulomatosa and Melkersson-Rosenthal syndrome are both rare and benign diseases. Because of their granulomatous character, a relationship to Crohn's disease has been suggested. Furthermore, because of their unknown aetiology, treatment is difficult, and evaluation of response is hampered by the natural tendency to spontaneous resolution and recurrence. OBJECTIVES AND METHODS To evaluate gastrointestinal involvement by clinical history, conventional endoscopy, and capsule endoscopy as well as to compare efficacy of treatment modalities on a series of 14 patients, one of the biggest collectives reported. RESULTS Four patients (4 of 14) were previously, simultaneously, or subsequently diagnosed with Crohn's disease. In six patients (6 of 14) with minor gastrointestinal symptoms as flatulence, occasional constipation, diarrhoea, or admixture of mucus with the stool, we could not detect any signs of inflammatory bowel disease by capsule endoscopy. Nine patients received clofazimine, and eight patients responded to treatment (four complete responses, four partial responses). Two patients were successfully treated with infliximab. Systemic methylprednisone was not successful in two patients. CONCLUSION Close to 30% of patients showed an association of cheilitis granulomatosa and Crohn's disease. Forty-three per cent of patients reported minor gastrointestinal irregularities without any detectable changes of Crohn's disease. Clofazimine seems to be an effective treatment, although long-term application is necessary with frequent aggravation in the beginning. Infliximab, an effective drug in Crohn's disease, could be a promising treatment option for severe cases.
منابع مشابه
Surgical treatment of persistent macrocheilia in patients with Melkersson-Rosenthal syndrome and cheilitis granulomatosa.
BACKGROUND Various conservative methods for treatment of labial swelling in patients with cheilitis granulomatosa have been attempted, often with only moderate success and sometimes with persistent disfiguring lip swelling. Severe macrocheilia can produce an unaesthetic facial deformity associated with functional disturbances. In patients with persistent macrocheilia, reduction cheiloplasty wit...
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Cheilitis granulomatosa is characterized by recurrent or persistent swelling of one or both lips. The exact etiology is not certain. It may represent oral manifestations of Crohn's disease and sarcoidosis, so the complete diagnostic work up of the patient with suspected Cheilitis granulomatosa is a must. Microscopically, a non-necrotizing granulomatous inflammation is the typical feature. Manag...
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Cheilitis granulomatosa is characterized by granulomatous lip swelling. We report a case of a 13-year-old girl who presented with orofacial swelling and arthralgia, who eventually was diagnosed with Crohn's disease, which was successfully treated with infliximab and azathioprine combination therapy. Recurrent or persistent orofacial swelling should prompt consideration of cheilitis granulomatos...
متن کاملMelkersson-Rosenthal syndrome: Appropriate response to the combination treatment with intralesional triamcinolone injection and oral azithromycin
Melkersson-Rosenthal syndrome is an uncommon disorder which is presented by a triad of lip swelling, facial nerve palsy, and a fissured tongue. A number of treatments have been reported for this syndrome with variable outcomes. We have reported a 38-year-old female patient with lower lip swelling and a fissured tongue on clinical examination. She was also suffering from facial nerve paralysis s...
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عنوان ژورنال:
- Journal of the European Academy of Dermatology and Venereology : JEADV
دوره 21 8 شماره
صفحات -
تاریخ انتشار 2007