https://orcid.org/0000-0003-2905-0979
Dear Editor,
The purpose of this letter is to highlight the efficacy of allergen avoidance and advocate for a more sensitive method for identifying these allergic triggers, in the treatment of patients suffering from granulomatous cheilitis.
Granulomatous cheilitis (GC) is a rare disorder characterized by chronic lip swelling due to noncaseating granulomatous inflammation (Citation1). GC may be idiopathic or associated with other granulomatous inflammatory disorders such as sarcoidosis or Crohn’s disease. The etiology of GC is still unknown with numerous possible mechanisms including genetic, infectious, and immunologic (Citation1–4). Of note, it has been widely reported that allergic reactions to various food substances and food additives may be either a causative or exacerbating factor in GC (Citation1–4). Both type 1 (immediate) (Citation3) and type IV (delayed) (Citation4) hypersensitivity reactions may play a role.
Unfortunately, there is currently no gold standard therapy for GC, and treatment is often difficult and unsatisfactory (Citation1,Citation4). Multiple topical, intralesional, and systemic therapies have been trialed — including antimicrobial agents, immunomodulatory and/or immunosuppressive drugs, and biologics — with varying degrees of success (Citation4). Systemic anti-inflammatory therapies can serve as short-term solutions to partially resolve the inflammatory symptoms of GC; however, many of these medications are often ineffective long-term therapeutic options with problematic adverse effects (Citation2).
One concern that the authors of this letter raise with current treatment options is that they attempt to broadly mitigate downstream inflammatory responses rather than focus on upstream preventative strategies. Based on our clinical experiences and a thorough review of the literature, we recommend that physicians and patients strongly consider allergen avoidance in their management of GC.
Anecdotally, some of our patients at the University of Utah who are on systemic immunosuppression for GC experienced fewer flares after avoiding common food and preservative allergens such as cinnamon, cacao, and sodium-benzoate. One patient, a 37-year-old female with a past medical history of biopsy-proven GC and diabetes, presented for orofacial swelling with prominent perioral edema that only partially responded to treatment with topical corticosteroids and tacrolimus, intralesional triamcinolone, and systemic TNF-alpha inhibitors. We proceeded with standard patch testing which revealed an allergy to cinnamates and recommended dietary avoidance. This dietary change reduced her orofacial edema significantly.
There are studies that have reported the efficacy of elimination diets alone, as a primary therapeutic regimen, without concurrent systemic immunosuppression. In a 2018 case report, a 13-year-old patient discontinued potent immunomodulatory treatments for four consecutive years after adhering to a diet devoid of cinnamon and benzoate derivatives (Citation5). In a prospective study of 25 GC patients evaluating cinnamon and benzoate elimination diets as first-line treatment, inflammation improved in 18 of 25 (72%) patients at both lip and oral sites after adhering to an 8-week elimination diet (Citation6). A review article found that a cinnamon- and benzoate-free diet improved symptoms in 54%–78% of patients with 23% requiring no adjunctive therapies (Citation7).
With anecdotal evidence and concrete data supporting the impact allergen avoidance can have on GC symptoms, it is essential to ensure our screening system is robust. Standard patch testing and contact urticarial testing are methods for identifying allergens involved in type IV and type I hypersensitivity reactions, respectively (Citation3). Patch testing appears to be the more commonly used method of identifying sensitivities in GC (Citation7), possibly because many large contact allergy clinics do not evaluate for contact urticaria (Citation8). As both type I and IV hypersensitivity reactions may be involved in GC, using only patch testing risks missing important allergens (Citation3). For example, patch testing has been shown to have a lower sensitivity for detecting cinnamon and benzoate sensitivities (55% vs. 7%, respectively) suggesting these compounds are primarily involved in a type I hypersensitivity reaction in GC patients (Citation3). Given the improvement in allergen detection that contact urticarial testing offers, the authors of this letter endorse its inclusion, in conjunction with standard patch testing, as a recommended GC screening protocol.
In summary, we humbly recommend adopting allergen avoidance as a main component of disease management for GC patients and support augmenting current allergen screening practices by utilizing both contact urticarial and standard patch testing.
Acknowledgement
The authors would like to acknowledge Dr. Steven R. Feldman for his contributions.
Disclosure statement
No potential conflict of interest was reported by the author(s).
Data availability statement
The data that support the findings of this study are openly available in the pubmed database.
Additional information
Funding
References
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