Allergy Capital

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Orofacial granulomatosis

Orofacial granulomatosis (OFG: also known as Melkersson-Rosenthal syndrome, Cheilitis Granulomatosis, and Schuermann's Glossitis Granulomatosa) is an uncommon inflammatory condition effecting the face and lips. People of all ages can be affected, but it is most common in the early adult years.

Lip swelling is the most common symptom
Lip swelling initially may only last a few hours at a time, and can be difficult to distinguish from another type of lip swelling known as angioedema. As the condition progress, swelling tends to last for days at a time, and eventually becomes permanent. Sometimes cracking and dryness of the lips occurs.

Other common symptoms
Swelling of the face and eyes also occurs. Some effected individuals have a "fissured tongue", and may sometimes develop facial paralysis. Mouth ulcers and inflammation of the gums, known as gingivitis, is sometimes seen. Other symptoms include tongue swelling or a sensation of a burning tongue.

Orofacial granulomatosis (OFG) is an inflammatory disease
Swelling and inflammation is seen in involved tissues, with epithelial hyperplasia, oedema, dilated lymphatics, lymphoid aggregates, and granulomas and fibrosis in late disease. While inflammation has been blamed on various infections from time to time, there is no definite proof that OFG is due to any one infectious organism. The cause of OFG is unknown.

Major & minor symptoms (table)

MAJOR SYMPTOMS	MINOR SYMPTOMS
lip swelling	burning tongue
inflammation of edges of mouth (angular chelitis)	altered taste
facial swelling	facial numbness/tingling
facial paralysis	spasm of eyelids (blepharospasm)
swelling of gums and cheeks	watery eyes
	runny nose, migraines

Orofacial granulomatosis showing fissured tongue (far left); thickened tongue (left of centre); chelitis/lip inflammation (right of centre); granulomatous inflammation (far right)

Confirming the diagnosis
As there are many possible causes of lip and facial swelling (see below), tests are often required to prove the diagnosis, and to exclude diseases that can mimic OFG. The tests selected will depend on clinical suspicions.

• Biopsy of involved tissue ­ to prove the diagnosis and exclude other conditions such as infection or tumour.
• Chest X-ray ­ helps to exclude sarcoid or tuberculosis/TB
• Blood Tests ­ helps to exclude Wegener's Granulomatosis, sarcoidosis
• Patch Testing ­ excludes contact sensitivity to ingested agents as a potential trigger
• Endoscopy/Colonoscopy tissue biopsy ­ excludes inflammatory bowel disease such as Crohn's
• Malabsorption Screen
• C1 Esterase
• Mantoux Test
• ANCA, ACE, calcium

Differential diagnosis of persistent facial swelling

• Infection ­ TB, Leprosy, Erysipelis
• Malignancy ­ Lymphoma, Leukaemia, Angiosarcoma
• Miscellaneous ­ Myxoedema, SVC obstruction
• Inflammation ­ Wegener's, Crohn's, OFG, Contact dermatitis, Rosacea, Sarcoid, Recurrent facial oedema with eosinophilia, Ascher's Syndrome (upper lip swelling, eye lid swelling and non-toxic goitre).
  

Differential diagnosis of lip swelling

Treatment
Sometimes swellings will resolve spontaneously without treatment, but most persist for many years. While no one treatment is always effective, a number of options are available. These include:

• Elimination diets
• Medications that reduce inflammation (cortisone/steroid tablets/injections, Methotrexate, Hydroxychloroquine, Sulfasalazine, some antibiotics)
• Radiotherapy
• Plastic Surgery
  

References

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