Case report of treating exfoliative cheilitis with interleukin-4 (IL-4) receptor alpha antagonist

Recently there is a case report published utilizing antagonist of interleukin-4 receptor alpha for a patient who is treatment-resistant to other typical treatment options for exfoliative cheilitis, namely topical corticosteroids, topical tacrolimus, petroleum jelly, and doxycycline.

Dupilumab, a human monoclonal antibody, functions as an antagonist for the interleukin-4 (IL-4) receptor alpha. By doing so, it hinders the pro-inflammatory IL-4 and IL-13 pathways. In the field of dermatology, dupilumab has gained approval for addressing moderate to severe atopic dermatitis, and more recently, prurigo nodularis.

The case is a 35-year-old woman with a history of atopic dermatitis since childhood who sought treatment at the clinic due to a persistent 2.5-year issue with erythematous and scaly lips. Monthly flares caused pain and pruritus, despite prior interventions by another dermatologist, including topical corticosteroids, topical tacrolimus, petroleum jelly, and doxycycline for possible concurrent rosacea. Although tacrolimus ointment prevented some flares, it did not reduce their duration. Previous negative test results, including patch testing and polymerase chain reactions for varicella zoster virus and herpes simplex virus, were noted.

Upon presentation at the clinic, the patient’s lips exhibited edema, scaling with microvesicles, and a few cream-colored pustules. Initial treatment included prescribing tacrolimus ointment for flare prevention, hydrocortisone valerate 0.2% ointment for flare management, prophylactic valacyclovir 500 mg twice daily for three months, and Vaseline as a lip barrier. While hydrocortisone alleviated flares, their frequency increased to weekly. Follow-up revealed persistent microvesicles and pustules on the lips. Dapsone 5% gel and crisaborole 2% ointment were ineffective, and systemic therapies like isotretinoin and methotrexate yielded no improvement. Alitretinoin was suggested but proved unaffordable.

Finally, dupilumab was prescribed with a 600 mg loading dose, followed by 300 mg every four weeks. After four months of treatment, the patient’s lips were no longer pruritic or painful. Topical tacrolimus and corticosteroids were no longer necessary, and examination showed resolved hyperkeratotic scale with no perioral lesions or scarring. Exfoliative cheilitis flares had ceased, with occasional eyelid irritation manageable with emollients.

This may be the first case report demonstrating successfully treating EC with dupilumab. Dupilumab, classified as a human monoclonal immunoglobulin-G4 (IgG4) antibody, specifically targets the shared IL-4Rα subunit, thereby blocking the IL-4 and IL-13 transduction pathways. This action is pivotal in inhibiting the aberrant T helper-2 (Th2)-mediated inflammatory pathway, contributing to the normalization of barrier function. By suppressing IL-4 and IL-13, dupilumab induces changes in gene expression, resulting in decreased markers of epidermal proliferation and inflammatory mediators. Additionally, it promotes an increase in lipid metabolism proteins, as well as structural and barrier proteins.

Exfoliative cheilitis manifests as inflammation and desquamation along the vermilion border of the lip, often accompanied by thick keratin scale. Histopathological examination reveals parakeratosis, hyperkeratosis, acantholysis, and both acute and chronic inflammation, including lymphoplasmacytic infiltration in the dermis. Notably, patients with EC demonstrate a predisposition to type I IgE-mediated hypersensitivity. Intriguingly, the patient in this case also had a history of childhood atopic dermatitis. It is plausible that the therapeutic impact of dupilumab, specifically in blocking the Th2 pathway, downregulating inflammatory mediators, and curbing epidermal proliferation, played a role in resolving EC for this individual. This case suggests that dupilumab could be a viable consideration for patients with resistant EC, particularly those with a history of atopic dermatitis.

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