OROFACIAL GRANULOMATOSIS

By Gary M. White, MD

orofacial granulomatosis Courtesy Michael O. Murphy, MD


Orofacial granulomatosis (OG) is a general term used to describe persistently swollen lips with granulomas seen histologically and no other associations.  The term Melkersson-Rosenthal syndrome is used when OG is seen in conjunction with facial palsy and fissured (scrotal) tongue. Allergic contact dermatitis to foods as well as Crohn's disease may cause a similar clinical picture.

Clinical

The lips are enlarged. It may be episodic at first, but eventually the swelling is becomes permanent. The upper lip is most commonly affected first, followed by the lower lip.

Differential Diagnosis

Various conditions may cause acute lip swelling, e.g. angioedema, infection, trauma, in contrast to MRS which is chronic. Ascher Syndrome is the combination of swelling of the upper lip and recurrent eyelid edema resulting in slack eyelid skin (blepharochalasis). Sarcoidosis and Crohn's disease may cause swelling of the lip with granulomas on biopsy.

Melkersson-Rosenthal Syndrome

See Melkersson-Rosenthal syndrome.

Allergic contact dermatitis

Some patients have noted worsening with certain foods. Allergy to potatoes, chocolate, cinnamic aldehyde, and monosodium glutamate has been found in some patients, and avoidance of such in the diet has led to improvement.  Also, allergy to toothpaste and metals in crowns or fillings (e.g. gold and mercury) has caused orofacial granulomatosis.

Cheilitis Granulomatosis

A term used to describe Crohn's disease affecting the lip.

Work-Up

Chest radiograph, PPD and/or interferon gamma should be performed to tuberculosis and sarcoidosis. Colonoscopy to exclude Crohn's disease and patch testing for food or other allergies is recommended.

Treatment

If an allergy to a food additive is found, dietary changes to avoid it should be made. If metal allergy is found, appropriate removal of fillings or crowns should be done. 

High-volume intralesional triamcinolone (e.g. 10 mg/ml) after nerve-block anesthesia has been recommended. It may be combined with oral metronidazole and/or oral minocycline. Two children were successfully treated with a combination of oral prednisolone and minocycline. One 12 year old girl was successfully treated with a 3-day course of intravenous corticosteroid pulses (dexamethasone at 1.5 mg/kg/d). The pulses were repeated once in 4 weeks and the patient received a total of six 3-day pulses. The patient was also chloroquine [PD Sept 17th 2017 online]

Alternatively, triamcinolone 40 mg/cc after prior treatment with an anesthetic cream has been successfully employed Br J Dermatol. 2014 Apr;170(4):794-801.

Azithromycin 500 mg daily x 3 consecutive days per week for 3 months cleared or nearly cleared 5 patients in one report [JAMA Derm 2015;151;219-20]. Thalidomide, hydroxychloroquine and clofazimine have been used. A 59-year-old woman nearly cleared after 6 months of mycophenylate 500 BID [JAAD 2014:70:e138].

Lip reduction surgery may be performed in cases recalcitrant to medical intervention.

Additional Pictures

It was difficult to tell in this patient between allergic contact dermatitis to food and orofacial granulomatosis.
Allergic contact dermatitis to food vs. orofacial granulomatosis

When seen in conjunction with facial palsy and fissured (scrotal) tongue, the term Melkersson-Rosenthal Syndrome is used.
Orofacial granulomatosis as part of Melkersson-Rosenthal Syndrome

References

A 43-year-old woman developed a permanently swollen upper lip for 16 months. She also reported burning and flushing of the face when nervous and occasional papules on the face and neck. There had been no regression in lip swelling since its onset. There was some improvement IL kenalog 10 mg/cc. Dermatology Online Journal 17(10).

A 30-year-old man with Down syndrome with gross swelling and deformity of the lower lip of seven years. Initially the swelling over the lower lip was episodic and recurrent in nature. However, for last three years, the swelling had tended to be persistent. Dermatology Online Journal 18(6)

A 15-year-old girl with painless swelling of upper and lower lips along with swelling of gums for the last 2 years. Contemp Clin Dent 2015;6, Suppl S1:94-6

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