Cheilitis glandularis

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ORPHA:1221OMIM:118330K13.0
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Overview

Cheilitis glandularis is a rare inflammatory disorder affecting the minor salivary glands of the lips, predominantly the lower lip. The condition is characterized by swelling, enlargement, and eversion of the lower lip due to inflammation, hyperplasia, and sometimes suppuration of the minor salivary glands. The ductal openings of these glands become dilated and prominent, and mucous secretions may be expressed from the lip surface. Cheilitis glandularis primarily affects middle-aged to older adults and has a male predominance. Three clinical subtypes have been historically described: simple type (characterized by multiple painless, pinpoint openings on the lip surface with mild swelling), superficial suppurative type (also known as Baelz disease, involving painless swelling with crusting and superficial bacterial infection), and deep suppurative type (involving deep-seated infection with abscess formation, fistulous tracts, and significant lip induration). Patients may experience chronic lip dryness, crusting, tenderness, and ulceration. The condition is considered a risk factor for the development of squamous cell carcinoma of the lip, particularly in the setting of chronic sun exposure, tobacco use, and poor oral hygiene. The exact cause of cheilitis glandularis remains unclear, though it is thought to be multifactorial, with contributing factors including chronic sun exposure (actinic damage), tobacco use, poor dental hygiene, and possibly genetic predisposition. Treatment depends on the severity and subtype. Conservative management includes lip balm with sun protection, avoidance of irritants, and topical corticosteroids. Antibiotics may be used for suppurative forms. In refractory or severe cases, surgical vermilionectomy (lip shave) is considered the definitive treatment and also serves to reduce the risk of malignant transformation. Regular follow-up is recommended due to the potential for squamous cell carcinoma development.

Clinical phenotype terms— hover any for plain English:

Squamous cell carcinomaHP:0002860Abnormal salivary gland morphologyHP:0010286
Inheritance

Multifactorial

Caused by a mix of several genes and environmental factors

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Cheilitis glandularis.

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No actively recruiting trials found for Cheilitis glandularis at this time.

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No specialists are currently listed for Cheilitis glandularis.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Cheilitis glandularis.

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Common questions about Cheilitis glandularis

What is Cheilitis glandularis?

Cheilitis glandularis is a rare inflammatory disorder affecting the minor salivary glands of the lips, predominantly the lower lip. The condition is characterized by swelling, enlargement, and eversion of the lower lip due to inflammation, hyperplasia, and sometimes suppuration of the minor salivary glands. The ductal openings of these glands become dilated and prominent, and mucous secretions may be expressed from the lip surface. Cheilitis glandularis primarily affects middle-aged to older adults and has a male predominance. Three clinical subtypes have been historically described: simple t

How is Cheilitis glandularis inherited?

Cheilitis glandularis follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Cheilitis glandularis typically begin?

Typical onset of Cheilitis glandularis is adult. Age of onset can vary across affected individuals.