Cheilitis glandularis

Definition

Cheilitis glandularis is a rare inflammatory disorder of the lip.

Epidemiology

Glandular cheilitis is a rare form of cheilitis, more prevalent in older males, but individuals in younger age and women also may be affected.

Cause

The cause is unknown, but several factors have been identified, including atopy, infection, chronic exposure to the sun, repeated licking, and use of tobacco.

Signs and symptoms

• It is mainly characterized by swelling of the lip with hyperplasia of the salivary glands; secretion of a clear, thick mucus; and variable inflammation.
• Enlargement and chronic exposure of the mucous membrane on the lower lip becomes affected by the environment, leading to erosion, ulceration, crusting, and, occasionally, infection.

Clinical presentation

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.

80%-99% of people have these symptoms

• Abnormal salivary gland morphology
• Abnormality of the salivary glands
• 0010286
• Abnormality of immune system physiology0010978
• Squamous cell carcinoma0002860
• Thick lower lip vermilion
• Increased volume of lower lip

Diagnosis

• The diagnosis of most cheilitis is based on clinical signs and a careful anamnesis. [1].
• The diagnosis of allergic contact cheilitis is confirmed by history of allergy and a relevant patch test reaction. Patients with negative patch testing are diagnosed with irritant contact cheilitis or atopic cheilitis.
• A biopsy is required in cheilitis granulomatosa to confirm the diagnosis.

Treatment

• The approach to treatment for cheilitis glandularis is typically based on information obtained from histopathologic analysis (microscopic examination of the tissue); the identification of the likely causes responsible for the condition; and attempts to alleviate or eradicate those causes.
• Given the relatively small number of reported cases of the condition, there is not sufficient or reliable data that exists with regard to medical approaches.
• Therefore, treatment generally varies accordingly for each individual.

• For cases attributable to angioedema (swelling similar to hives beneath the skin), an antihistamine may help with temporary reduction of acute, nonpurulent (lacking pus) swelling.
• Suppurative cases (those with pus present) typically require management with appropriate antimicrobial treatment as determined by culture and sensitivity testing.
• Concomitant corticosteroid treatment may increase the effectiveness of antimicrobial therapy in cases with nodularity; however, the potential adverse effects of long-term corticosteroid treatment, and because it can promote local fibrosis and scarring, limit its potential use either as an adjunct to antibiotic treatment or as a single therapeutic modality.
• Topical 5-fluorouracil is useful for treatment of dysplastic actinic cheilitis and to curtail its progression.
• In conjunction with clinical supervision, it can be prescribed as an alternative to vermilionectomy (sometimes called a lip shave) or as a preventative measure following vermilionectomy.
• In cheilitis glandularis cases in which a history of chronic sun exposure exists (especially if the individual is fair skinned or the everted lip surface is chronically eroded, ulcerated, or crusted), biopsy is strongly recommended to rule out actinic cheilitis or carcinoma.
• Surgical excision is typically not necessary when the diagnosis is actinic cheilitis with atypia or only mild dysplasia; however, individuals require ongoing clinical vigilance at regular intervals and instruction in measures to protect the lips from further sun damage.
• Treatment options for cases of actinic cheilitis with moderate-to-severe dysplasia include surgical stripping or vermilionectomy, cryosurgery or laser surgery, or topical chemotherapy with 5-fluorouracil.
• Given the potential for recurrence and the risk for development of carcinoma, sun protective measures and regular clinical monitoring should be instituted.
• In cases in which eversion, extensive fibrosis, and induration have resulted in lip incompetence with functional and cosmetic compromise, chronic pain, and surface disruption, surgical cheiloplasty (lip reduction) may be indicated to restore normal lip architecture and function.
• Cheiloplasty is also a prophylactic measure for reducing the risk of actinic injury.

References

Media related to Cheilitis glandularis at Wikimedia Commons

NIH genetic and rare disease info

• NIH info on Cheilitis glandularis

Cheilitis glandularis is a rare disease.

Cheilitis glandularis Resources
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