Cheilitis granulomatosa: overview of 13 patients with long-term follow-up--results of management.
BACKGROUND
Cheilitis granulomatosa, often regarded as a subtype of orofacial granulomatosis, is characterized by recurrent or persistent swelling of one or both lips. Classically, a non-necrotizing granulomatous inflammation is seen at histologic examination. Although a relationship has been proposed between Melkersson-Rosenthal syndrome (and the monosymptomatic form, cheilitis granulomatosa) and Crohn's disease on the basis of the orofacial swelling and similar histology, several studies of Melkersson-Rosenthal syndrome have not found an association with Crohn's disease.


METHODS
The clinical features, histopathology, association with Crohn's disease, and results of nonsurgical and surgical therapy in 13 patients with cheilitis granulomatosa were investigated in a retrospective case study with a mean follow-up period of 8.2 years.


RESULTS
There was a low chance of developing Crohn's disease. Most patients in this study responded to nonsurgical treatment modalities. Patients with deterioration of lip swelling usually responded to intralesional injections with triamcinolone or to short courses of systemic glucocorticoids. Nonsteroidal systemic modalities, such as clofazimine, hydroxychloroquine, or sulfasalazine, were alternatives to glucocorticoid regimens, thus avoiding the long-term side effects of corticosteroids. Surgical intervention should only be performed in severely disfiguring cases.


CONCLUSIONS
The management of cheilitis granulomatosa remains a challenge. As this study revealed a low chance of developing Crohn's disease, it does not seem justified to inform patients with cheilitis granulomatosa of the possibility that they might develop Crohn's disease. Patients with a negative history of gastrointestinal complaints should not be exposed to routine investigations of the gastrointestinal tract.
van der Waal
Rutger I F
RI
Department of Dermatology, University Hospital Vrije Universiteit/ACTA, PO Box 7057, 1007 MB Amsterdam, the Netherlands. rif.vdwaal@vumc.nl
Schulten
Engelbert A J M
EA
van der Meij
Erik H
EH
van de Scheur
Martijn R
MR
Starink
Theo M
TM
van der Waal
Isaäc
I
D000293:Adolescent; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage*; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage*; D001707:Biopsy, Needle; D002990:Clobetasol / Q000008:administration & dosage; D002991:Clofazimine / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008046:Lip / Q000503:physiopathology; D008297:Male; D008556:Melkersson-Rosenthal Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology*; D008875:Middle Aged; D009426:Netherlands; D011239:Prednisolone / Q000008:administration & dosage; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D000894:Anti-Inflammatory Agents, Non-Steroidal; D011239:Prednisolone; D002990:Clobetasol; D002991:Clofazimine