The Case of Swollen Lips

I got an interesting consult the other day. A 29-year-old man reported to the oral surgery clinic with a complaint of swollen lips. The lips had been swollen for over 5 years, he had seen numerous doctors and according to him tried every single remedy under the sun. He was currently using a low dose of corticosteroids but they were not working. 

The man appeared to be in great health otherwise. 

Orofacial granulomatosis (courtesy Dr. Asma Pervez)

The labial swelling was firm on palpation and there was no pain associated with it. During the intra-oral exam, gingival hyperplasia was noted anteriorly. 

Orofacial granulomatosis (Courtesy Dr. Asma Pervez)

The tissue from both sites was biopsied and submitted for histopathologic examination. The tissue from the lip showed presence of some acute and chronic inflammatory cells. The specimen from the gingiva exhibited granulomatous inflammation. Because we can assume that both changes are part of the same process, we concluded that the patient most likely has oro-facial granulomatosis.

Intra-lesional steroid injections were recommended as management. I have not heard back from the surgeon yet about results. I'll update the post once the results become available. 

Oro-facial Granulomatosis:

Oro-facial granulomatosis is an abnormal immune response that presents as swelling on the lips and tissues in the anterior region of the mouth.  Additional features, such as linear vestibular ulcerations, "bumpy" edema of oral tissues known as cobblestoning and hyper plastic tissue folds may also be encountered. The condition manifests in the third and forth decades of life. The condition is currently characterized as idiopathic. A dietary link has been suggested but there is very little evidence to support this claim. Histopathologic examination exhibits granulomatous inflammation which may not be evident in the first section. Several sections may be needed to confirm the presence of granulomas.

Oro-facial granulomatosis brings a couple of things in the differential diagnosis. Clinical and histopathologic changes of oro-facial granulomatosis are identical to those of Crohn's disease. We confirmed absence of intestinal disease to rule out this possibility. This step usually requires referral to a good gastroenterologist.

Melkersson-Rosenthal syndrome is a triad of oro-facial granulomatosis, fissured tongue and facial paralysis. Since the later two were absent in our patient, this was an easy possibility to rule out.