eMedicine Specialties > Dermatology > Diseases of the Oral Mucosa

Cheilitis Glandularis

Author: Ellen Eisenberg, DMD, Professor, Section Chair and Director, Oral and Maxillofacial Pathology, Oral Pathology Biopsy Service, Department of Oral Health and Diagnostic Sciences; Associate Professor, Division of Anatomic Pathology, Department of Pathology and Laboratory Medicine, University of Connecticut Health Center
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Updated: Dec 31, 2008

Introduction

Background

Cheilitis glandularis (CG) is a clinically descriptive diagnosis that refers to an uncommon, poorly understood, and fundamentally benign inflammatory disorder of the submucosal glands in the lower lip. Its etiology remains obscure.¹ The condition is characterized by progressive enlargement and eversion of the lower labial mucosa that results in obliteration of the mucosal-vermilion interface. With externalization and chronic exposure, the delicate lower labial mucous membrane is secondarily altered by environmental influences, leading to erosion, ulceration, crusting, and, occasionally, infection. Most significantly, susceptibility to actinic damage is increased. Therefore, cheilitis glandularis can be considered a potential predisposing factor for the development of actinic cheilitis and squamous cell carcinoma.

Historically, the disorder has been subclassified into 3 types: simple, superficial suppurative, and deep suppurative. The deep suppurative type has also been variously referred to as myxadenitis labialis or cheilitis apostematosa, and the superficial suppurative type has been termed Baelz disease. Many believe these subtypes represent a continuum of disease, where the simple type, if not treated, could become secondarily infected and progress to become the superficial or eventually, the deeply suppurative type.

Pathophysiology

In 1870, von Volkmann introduced the term cheilitis glandularis.² He described a clinically distinct, deeply suppurative chronic inflammatory condition of the lower lip characterized by mucopurulent exudates from the ductal orifices of the labial minor salivary glands. In 1914, Sutton proposed that the characteristic lip swelling was attributable to a congenital adenomatous enlargement of the labial salivary glands.^3,4 This remained the prevailing hypothesis until 1984 when Swerlick and Cooper reported 5 new cases and a retrospective analysis of all cases of cheilitis glandularis reported up to that time.⁵ Their studies revealed no evidence to support the assertion that submucosal salivary gland acinar hyperplasia is either responsible for or an actual feature of established cheilitis glandularis.

Since then, other authors have corroborated this observation and have variously reported salivary ductal ectasia, hyperplasia, and squamous metaplasia. Stoopler et al reported a papillary cystadenoma-like ductal growth pattern in one patient with cheilitis glandularis.⁶ Periductal chronic inflammation (dochitis), scarring, and chronic sclerosing sialadenitis in otherwise unremarkable minor salivary glands have also been noted. Musa and coauthors reported a case with a more generalized presentation of cheilitis glandularis, which they termed suppurative stomatitis glandularis.⁷ In their patient, suppurative labial minor salivary gland involvement extended to also involve the buccal mucosa. Reichart and coauthors reported a retention cyst in the upper lip of an elderly patient with the "simplex" form of the condition; they interpreted the retention cyst as a consequence of cheilitis glandularis. However, this is mere conjecture on the authors' part.⁸

In a case reported by Leao and coauthors, cheilitis glandularis was the presenting clinical finding in a patient later discovered to have undiagnosed HIV-infection.⁹ The lip findings could have resulted from (undiagnosed but HIV-related) minor salivary gland dysfunction leading to exfoliative cheilitis, a well-documented and characteristic manifestation of HIV-infection. Butt and coauthors reported a case of cheilitis glandularis that progressed to squamous cell carcinoma in an HIV-infected patient.¹⁰

Although some have speculated that cheilitis glandularis represents a hereditary autosomal dominant condition, composite findings in most cases appear to indicate the condition represents a clinical reaction pattern to chronic irritation of the lip from various highly diverse external causes, which include actinic damage, factitial injury, atopy, infection, and tobacco irritation. Carrington and Horn report a case in which an elderly man developed cheilitis glandularis related to actinic damage following vermilionectomy for squamous cell carcinoma of the lower lip.¹¹ These authors advocate clinical investigation in cases of cheilitis glandularis to rule out neoplastic, immune suppressive, or inflammatory changes due to local factors. This illustrates the concept that cheilitis glandularis is not a separate and distinct disease. Instead, it appears to be a descriptive phenomenon that could represent any one of a host of diverse clinicopathological entities.

The possibility of a genetic predisposition for cheilitis glandularis has been raised by some authors. Parmar and Muranjan, among others, described a genetic syndrome involving "double lip" of both lips in conjunction with ptosis and other physical abnormalities.^12,13  Dhanapal and coworkers reported a case in which a 14-year-old girl with double lip developed cheilitis glandularis.¹⁴

Frequency

United States

This is an uncommon condition in the United States. However, factitial injury to the lower lip vermilion border and mucosa due to chronic lip biting, lip drying, or habitual lip licking, combined with sun-induced lip damage, are extremely commonplace. Any of the latter influences could result in lip eversion, ulceration, and secondary infection.

International

Similar to its prevalence in the United States, the condition is uncommon worldwide

Mortality/Morbidity

Cheilitis glandularis has been associated with a heightened risk for the development of squamous cell carcinoma. In many cases, dysplastic (premalignant) surface epithelial change is evident histopathologically, and frank carcinomas have been reported in 18-35% of cases. Rarely, cases of chronic persistent or recurrent suppurative infection may result from inappropriate antibiotic treatment.

A case of persistent suppurative cheilitis glandularis, confirmed by punch biopsy, in a 52-year-old African American woman with a 15-pack year smoking history responded to a 4-week course of oral penicillin at 1 g/d combined with oral fluoroquinolone at 1 g/d. Two weeks into the therapy, the swelling was significantly reduced. The antibiotic regimen was continued for 2 additional weeks, with resolution of the lip lesions and continued normality at 1-year follow-up.¹⁵

Race

Cheilitis glandularis has no apparent racial predilection. However, actinic damage is more common in whites than in darker-skinned individuals.

Sex

The disorder appears to favor adult males; however, cases have been reported in women and in children. One possibility is that some purported childhood cases actually represent exfoliative cheilitis, frequently attributable to factitial injury. Actinic cheilitis favors adults in midlife or older, and a male predilection is reported for the latter condition.

Age

The condition most frequently occurs between the fourth and seventh decades of life; however, the age range is wide. One report exists of cheilitis glandularis in a child, and several cases have arisen in teenagers and young adults.

The risk of dysplasia and carcinoma increases with age, especially in fair-skinned individuals with sun-damaged skin. This is because the characteristic eversion of the lower lip results in long-term chronic exposure of the thinner, more vulnerable labial mucosa to actinic influence.

Clinical

History

Cheilitis glandularis is a chronic progressive condition. Patients typically present for diagnostic consultation within 3-12 months of onset. Complaints vary according to the nature and the degree of pain, the enlargement and the loss of elasticity of the lip, and the extent of evident surface change.

• Asymptomatic lip swelling initially occurs with clear viscous secretion expressible from dilated ductal openings on the mucosal surface.
• Some patients report periods of relative quiescence interrupted by transient or persistent painful episodes associated with suppurative discharge.
• A burning discomfort or a sensation of rawness referable to the vermilion border may be reported. This is associated with atrophy, speckled leukoplakic change, erosion, or frank ulceration with crusting.

Physical

• Cheilitis glandularis affects the lower lip almost exclusively. It manifests as progressive, often multinodular enlargement, eversion, and induration.
• Salivary gland duct orifices may be dilated and appear as red or black puncta.
• Viscous clear secretions may initially exit the duct openings spontaneously.
• In more suppurative cases, application of gentle pressure can elicit mucopurulent exudates.
• With advancing lip prominence and mucosal eversion, the mucosal-vermilion junction is obfuscated.
• Prolonged exposure to the external environment results in desiccation and disruption of the labial mucous membrane, predisposing it to inflammatory, infectious, and actinic influences.
• Cheilitis glandularis had historically been subclassified into 3 types, now believed to represent evolving stages in severity of a single progressive disorder.
  • In the simple type, multiple, painless, papular surface lesions with central depressions and dilated canals are seen.
  • The superficial suppurative type (also referred to as Baelz disease) consists of painless, indurated swelling of the lip with shallow ulceration and crusting.
  • Cheilitis glandularis of the deep suppurative type (cheilitis glandularis apostematosa, cheilitis glandularis suppurativa profunda, myxadenitis labialis) comprises a deep-seated infection with formation of abscesses, sinus tracts and fistulas, and potential for scarring.
  • The latter 2 types of cheilitis glandularis have the highest association with dysplasia and carcinoma, respectively.

Causes

Cheilitis glandularis is an unusual clinical manifestation of cheilitis that evolves in response to one or more diverse sources of chronic irritation.

• Lip enlargement is attributable to inflammation, hyperemia, edema, and fibrosis.
• Surface keratosis, erosion, and crusting develop consequent to longstanding actinic exposure or unusual repeated manipulations that include self-inflicted biting or other factitial trauma, excessive wetting from compulsive licking, drying (sometimes associated with mouth breathing, atopy, eczema, and asthma), and any other repeated stimulus that could serve as a chronic aggravating factor.

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