Cheilitis granulomatosa and Melkersson-Rosenthal syndrome: evaluation of gastrointestinal involvement and therapeutic regimens in a series of 14 patients.
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.


OBJECTIVE
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.


CONCLUSIONS
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.
Ratzinger
G
G
Department of Dermatology and Venereology, Medical University, Innsbruck, Austria. gudrun.ratzinger@uibk.ac.at
Sepp
N
N
Vogetseder
W
W
Tilg
H
H
D000328:Adult; D000368:Aged; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D053704:Capsule Endoscopy; D002991:Clofazimine / Q000627:therapeutic use; D003424:Crohn Disease / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology*; D016099:Endoscopy, Gastrointestinal; D005260:Female; D005765:Gastrointestinal Agents / Q000627:therapeutic use; D005767:Gastrointestinal Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology*; D006801:Humans; D000069285:Infliximab; D008297:Male; D008556:Melkersson-Rosenthal Syndrome / Q000150:complications*; D008875:Middle Aged; D016896:Treatment Outcome
D000894:Anti-Inflammatory Agents, Non-Steroidal; D000911:Antibodies, Monoclonal; D005765:Gastrointestinal Agents; D000069285:Infliximab; D002991:Clofazimine