INTRODUCTION
Cheilitis granulomatosa (CG) is a subtype of orofacial granulomatosis that results in facial and oral swelling in the absence of non-caseating granulomatous systemic diseases such as Crohn’s and sarcoidosis.1 With concurrent facial palsy and a plicated tongue, the condition is referred to as Melkersson-Rosenthal syndrome.1,2 Both CG and Melkersson-Rosenthal syndrome are in the orofacial granulomatosis family of diseases.1 CG has no reported association with race or sex and is most commonly found in young adults.2 The initial phase of the disease often manifests as intermittent swelling of the upper lip. Over time, the symptoms become persistent and may involve both lips, resulting in functional disability and cosmetic disfigurement.3 The condition is typically painless. While there is no definitive treatment of CG, intralesional corticosteroids are widely used.1
CASE
We would like to report the case of a 27-year-old Caucasian woman with a long history of ulcerative colitis (confirmed to not be Crohn’s by multiple annual colonoscopies) and psoriasis vulgaris who presented to an outside dermatology office with edema of her bilateral lips, oral mucosal ulcerations, cheek and jaw swelling, and regional cervical lymphadenopathy. The patient at this time was on adalimumab for her ulcerative colitis. A skin biopsy of her lip revealed collections of histiocytes consistent with an early phase of cheilitis granulomatosa. She was treated with six days of methylprednisolone but had only marginal improvement. She was then instructed to use topical tacrolimus twice daily on her lips.
After two months without much improvement, however, the patient came to our clinic for a second opinion. Upon physical examination there was marked edema of her lower lip (Figure 1). With approval from her gastroenterologist, we switched her from adalimumab to certolizumab 400mg every two weeks.
After three months of treatment, she had significant improvement (Figure 2) and was back to her baseline level of lip fullness and no longer suffered from any oral ulcerations or lymphadenopathy. The patient maintained this improvement for six months but was then changed over to ustekinumab at the request of her gastroenterologist to better control her ulcerative colitis. She has since remained stable and in remission from her cheilitis granulomatosa on ustekinumab for over a year.
Cheilitis granulomatosa is a rare, painless, idiopathic swelling of the lip (or lips), found in only 0.08% percent of the general population.2 While orofacial granulomatosis can conceal underlying Crohn’s disease,4 our patient had had a recent colonoscopy and many prior confirming her disease was consistent with ulcerative colitis. There is no standard treatment for CG and its mechanism of disease is not well understood. It is speculated that a random influx of inflammatory cells is responsible, rather than a specific antigen.1 CG is often treated with oral, intralesional or topical corticosteroids, but other agents including metronidazole, tetracyclines, clofazimine, roxithromycin, and adalimumab have been used. Since infiltration of the lip with inflammatory cells occurs in CG, the anti-TNF agent certolizumab was initially considered for
