What is Causing This Woman’s Fat Lip?
A 47-year-old African American female presents to her primary care physician with a 1- month history of a persistent swollen left lower lip. She is concerned about her appearance because she will soon be departing for vacation on a cruise ship. She had visited the emergency room 2 weeks prior and had been given diphenhydramine and prednisone 60 mg for 5 days, but failed to respond as she had previously.
History
She denied any history of trauma or recent dental work, but reports multiple previous episodes of angioedema over the past 7 years. There was no obvious single recurrent precipitating factor, although she identified episodes temporally associated with a sandstorm—salt ingestion, sushi, taco sauce, and sun exposure.
She had never been prescribed an angiotensin-converting enzyme inhibitor, angiotensin receptor blocker, or calcium channel blocker, and had recently discontinued her hydrochlorothiazide. Her past medical history was remarkable only for prehypertension.
Physical Examination
Her physical examination revealed a very healthy and pleasant female with a 2 cm localized swollen left lower lip (Figure 1). Vital signs were normal. Her voice was normal and there were no signs of respiratory distress. There were no other facial or oral abnormalities noted. Lungs were clear and skin exam was unremarkable.
(Answer and discussion on next page)
Answer: Granulomatous cheilitis
Laboratory tests. Her evaluation revealed normal laboratories measuring complete blood count, liver function tests, basic metabolic panel, thyroid-stimulating hormone, erythrocyte sedimentation rates/C-reactive protein, urinanalysis, complement studies, ACE, and a tryptase level. A dental exam was unremarkable.
She was prescribed a course of oral corticosteroids, H1 and H2 receptor antihistamines, and referred for an allergy consultation.
Diagnosis. The allergist clinically diagnosed her with granulomatous cheilitis, which was confirmed by biopsy (Figure 2). A subsequent work-up for granulomatous diseases was ordered to include quantiferon for tuberculosis and colonoscopy and to exclude a diagnosis of Crohn’s; the results were unremarkable.
Figure 1. Patient presents with lip swelling.
Outcome of the case. The patient was advised to avoid triggers and given cetirizine and montelukast, a burst of corticosteroids followed by intralesional steroids. Since that time the patient has had some relief with avoidance of known triggers.1
Discussion. Granulomatous cheilitis (or cheilitis granulomatosa) is a rare inflammatory disorder. It is also called Miescher’s syndrome when occurring alone or as Melkersson-Rosenthal syndrome if associated with facial palsy of plicated or fissured tongue.2
Figure 2. Low power (40X) photomicrograph of a full thickness lip biopsy showing superficial dermal chronic inflammation in addition to mid and deep dermal granulomatous inflammation (left) and high power (200X) photomicrograph reveals a well-formed non-necrotizing granuloma surrounded by a rim of chronic inflammatory cells (right).
Granulomatous cheilitis primarily affects young adults but can occur at any age and may be associated with sarcoidosis, Crohn’s disease, infection, or foreign bodies. The usual course is one of intermittent acute episodes of painless lip swelling that eventually progress to a chronically swollen lip. It may involve either or both lips. Initial episodes may last hours to days. Recurrences can range from days to years leading to persistent symptoms.
Many different treatments are described in the literature, but none stand out as universally effective and reliable. Corticosteroids are a cornerstone of modern therapy (topical, intralesional are preferred), but there are other intriguing treatment options for refractory cases including infliximab a monoclonal antibody specific for tumor necrosis factor alpha or surgical correction.
Clinicians should keep a broad differential when seeing patients as atypical manifestations of common conditions as this may signal a different condition than initially expected. Any patient who has unresolving angioedema should undergo a lip biopsy to rule out granulomatous cheilitis.
Hear details from the case's author in the podcast below:
References:
- Banks T, Gada S. A comprehensive review of current treatments for granulomatous cheilitis. Br J Dermatol. 2012;166(5):934-937.
Agarwal N, Agarwal K, Prasad RK. Miescher’scheilitis granulomatosa a form of Melkersson-Rosenthal syndrome. Int J Dent Clin. 2011;3(2):113-114.