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Concurrent anomalies: Cheilitis glandularis and double lip
Concurrent anomalies: Cheilitis glandularis and double lip Report of a case Uonald M. Cohen, DMD, MS,* James G. Green, DDS,** Scott L. Diekmann, DDS,*** Lincoln, Neb. UNIVERSITY
OF NEBRASKA
MEDICAL
CENTER
COLLEGE
OF DENTISTRY
Double lip and cheilitis glandularis are uncommon anomalies unique case of concurrent double lip and cheilitis glandularis and treatment of both lesions are reviewed. Their concurrent unrelated. (ORAL SURC ORAL MED ORAL PAIXIOL 1988;66:397-9)
D ouble lip and cheiiitis gianduiaris are both uncommon anomalies of the lips. Double lip is caused by excessive areolar tissue and noninflammatory labial mucous gland hyperplasia of the pars villosa.’ When the lip is tensed, the pars villosa sagsbelow the pars glabrosa. Cheilitis glandularis is considered to be an inflammatory hyperplasia of the lower labial salivary glands.* A unique case of concurrent double lip and cheilitis glandularis is described, and the recognition and treatment of these entities is discussed. CASE REPORT
A 65year-old white man was seen at the clinic for routine dental care. During the initial examination, bilateral redundant folds of soft tissue were noted on the mucosal surface of the upper lip. When the upper lip was tensed, the redundant tissue became evident and took the shape of a “Cupid’s bow.” The lower lip was enlarged and everted and had numerous nodular swellings. The surface was diffusely leukoplakic and dotted with several dilated secretory ducts. A thick mucoid secretion could be expressed as a result of manipulation. The patient indicated that the lower lip had occasionally become painful and clear fluid had been present overlying the inflammed secretory ducts. The lower lip was somewhat inelastic and *Associate Professor, Department of Pathology, Diagnosis, and Radiology, University of Nebraska Medical Center College of Dentistry. **Assistant Professor and Director, General Practice Residency, Department of Pathology, Diagnosis, and Radiology, University of Nebraska Medical Center College of Dentistry. ***In Private Practice, Conifer, Colo.
and
involving the minor salivary glands of the lip. A is presented. The development, diagnosis, development in this patient appears to be
The patient was fair in complexion and had a long history of exposure to the sun. He had spent considerable time in the U.S. Navy and had farmed for the past 20 years. He had been aware of his upper double lip since childhood and could not recall any other family member with a similar condition. The lesion on the lower lip had been present for several years, and he first noticed it after several severe episodes of sunburned lips. He denied having any infection and could not recall any family member who was similarly affected. Surgical treatment was recommended for both lip lesions. Even though the patient was inform4 of the high risk of squamous cell carcinoma associated with cheilitis glandularis, he remained unconcerned about the lip swellings and thought that he was too old to be worried about cosmetics. He agreed to wear a hat and use sunscreen to protect his lips when working outside. The patient’s course has been followed for 8 years, and he has had no further clinical changes in the lower lip. DISCUSSION
Double lip is a congenital or acquired oral anomaly with no known gender predilection. The congenital form is thought to develop from persistence of the horizontal sulcus between the pars glabrosa and the pars villosa during the development of the lip.3 Acquired forms, which develop later in life, have been described as occurring after trauma or in association with Ascher’s syndrome (blepharochalasis and double lip with or without nontoxic goiter).‘*4 Clinically, double lip consists of a fold of excess or 397
398 Cohen, Green, and Diekmann
Oral Surg September19X8
Fig. 1. Clinical photograph demonstrating upper double lip with lip tensed and cheilitis glandularis of lower lip. The lower lip is enlarged and everted and there are several dilated secretory ducts visible on the mucosal surface.
redundant
tissue on the mucosal side of the lip.
Although it most often occurs bilaterally on the upper lip, it may be unilateral and can affect either or both of the lips simultaneously.4-8 While the condition is not evident with the upper lip at rest, the excess or redundant tissue will project beyond the vermilion border of the lip and take the form of a “Cupid’s bow” when the upper lip is tensed.6On the lower lip, unilateral or bilateral bulbous masseswill project intraorally.6 The condition is usually present at birth but does not become apparent until the permanent teeth erupt.’ Treatment of double lip is surgical and becomes necessary for cosmetic reasons or if it interferes with speechor mastication. 6*lo The surgical procedure can be done on an outpatient basis with the use of bilateral infraorbital blocks for the upper lip and mental blocks for the lower lip. These are the preferred methods of administering anesthesia since they avoid distortion of the surgical area by infiltration.” Acquired double lip has been reported to recur after surgery, but this has not been observed with the congenital form.‘* Cheilitis glandularis is a disease of the lower lip characterized by hyperplasia of the minor salivary
glands with varying degrees of inflammation. The condition occurs primarily in adult men, but cases have been reported in women and children.‘3~‘4The exact etiology of cheilitis glandularis is unknown, although congenital predisposition and familial inheritance,13*I5 emotional disturbances,14 bacterial infection,* poor oral hygiene,16and chronic irritation from wind, sun, chemicals, and tobacco” have all been advanced as causes. Cheilitis glandularis simplex is the most common of the three clinical forms and develops as a nodular enlargement that produces eversion of the lip.13The secretory ducts become dilated and inflammed, appearing as small red or white macules on the mucosal surface.‘8 A viscid mucous secretion may ooze from them or be expressed on palpation. The superficial type (Baelz’s disease) and deep suppurative type (cheilitis glandularis apostematosa,or myxadenitis, labialis) represent progressive stages of bacterial involvement, inflammation, and enlargement of the lip. I* Eventually, deep ulceration with abscess formation and fistulous tracts may develand the lip may become two to three times op, ‘3*18*19 its normal size.‘) Scarring, secondary to persistent inffammation’and infection, causesthe Iip to become
Cheilitis
Volume 66 Number 3
inelastic.” Although the condition was originally described as painless, pain may be a variable feature of all three types and is thought to be caused by bacterial sialadenitis and mucositis.18 Cheilitis glandularis has been associated with an increased risk for the development of squamous cell carcinoma. Dysplasia has been consistently found in most casesof cheilitis glandularis, and squamous cell carcinoma has been reported to develop in 18% to 35% of the cases.Za*21 Hyperplasia of the labial salivary glands causes eversion of the lower lip, exposing the thinner, more susceptible inner labial mucosa to actinic radiation.20 Surgery is the preferred treatment for cheilitis glandularis because of the risk of dysplasia and squamous cell carcinoma.**I83** Vermilionectomy with or without lip reduction has provided satisfactory results. Antibiotics, radiation therapy, and corticosteroids have not produced consistent or sustained relief.18 SUMMARY A unique case of concurrent upper double lip and cheilitis glandularis of the lower lip has been presented, and the factors involved in the development, pathogenesis, diagnosis, and treatment of these uncommon lesions have been reviewed. Surgery is the recommended treatment modality, particularly for cheilitis glandularis because of the inherent increased risk of malignancy. The concurrent double lip and cheilitis glandularis in this patient are most likely unrelated. REFERENCES 1. Gorlin RJ, Pindborg JJ, Cohen MM. Syndromes of the head and neck. New York: McGraw-Hill, 1976:253-S. 2. Doku HC, Shklar G, McCarthy PL. Cheilitis glandularis. ORAL SURG ORAL MED ORAL PATHOL 1965;20:563-7 1. 3. Warbick JG, McIntyre JR, Ferguson AG. Remarks on the aetiology of congenital bilateral fistulae of the lower lip. Br J Plast Surg 1952;4:254-62. 4. Barnett ML, Bosshardt LL, Morgan AF. Double lip and
glandularis
and double lip
399
double lip with blepharochaiasis
(Ascher’s syndrome). ORAL
SURC ORAL MED ORAL PATHOL
1972;34:727-33.
5. Stein R. Blepharochalasis des Unterlides. Klin Monatsbl f Augenh 1930;84:846-5 1. 6. Hausamen JE, Solbach HG, Pape HD. Klinischer Beitrag zum Ascher-Syndrom. Dtsch Zahnaerztl Z 1969;24:983-7. 7. Delaire J, Landais H, Billet J, Lajarte AY. Les doubles l&es. Actual Odontostomat 1969;23:365-80. 8. Dingman RO, Billman H. Double lip. J Oral Surg 1947; 5: 146-48. 9. Converse JM. Plastic and reconstructive surgery. 2nd ed. Philadelphia: WB Saunders, 1977:1543. 10. Oldfield MC. Prolapse of the lip mucosa. Br J Surg 1959;47:58-60. . II. Schwimmer A, Dymk H, Barr C. Surgical repair of a double lin. J Am Dent Assoc 1979;99:993-4. 12. Rintila AE. Congenital double lip and Ascher syndrome. II. Relationship to the lower lip sinus syndrome. Br J Plast Surg 1981;34:31-4. 13. Weir TW, Johnson WC. Cheilitis glandularis. Arch Dermatol 1971;103:433-7. 14. Woodburne AR, Philpott OS. Cheilitis glandularis: a manifestation of emotional disturbance. Arch Dermatol Syph 1950;62:820-8. 15. Sutton RL. Cheilitis glandularis apostematosa. J Cutan Dis 1909;27: 150-5. 16. Volkmann R. Einize Falle von Cheilitis glandularis apostematosa (Myxadenitis labialis). Arch Path01 Anat 1870; 50: 142-4. 17 I I. Eve:ett FG, Ho!der T?. Chei!itis glandularis apostematosa. ORAL SURC ORAL MED ORAL PATHOL 1955;8:405-13. ID, Pickett AB. Cheilitis glandularis. ORAL ORAL MED ORAL PATHOL 1980;49:526-9.
18. Oliver
SURG
19. Shafer WG, Hine MK, Levy BM. A textbook of oral pathology. 3rd ed. Philadelphia: WB Saunders, 1974:17-8. R. Cheilitis nlandularis. Heterotonic salivary 20. Michalowski glands and squamous cell carcinoma of the lip. Br’J Dermatol 1962;74:445-9. 21. Touraine A. Les cheilites glandulaires et leur cancer. Presse Med 1950;58:1369-70. 22. Brit BD. The “lip shave” operation for premalignant conditions and microinvasive carcinoma of the lower lip. J Otolaryngol 1977;6:407- I 1. Reprint requests IO: James G. Green, D.D.S. Assistant Professor and Director Department of Pathology, Diagnosis and Radiology University of Nebraska Medical Center College of Dentistry 40th and Holdrege Lincoln, NE 68583-0740
OF NEBRASKA
MEDICAL
CENTER
COLLEGE
OF DENTISTRY
Double lip and cheilitis glandularis are uncommon anomalies unique case of concurrent double lip and cheilitis glandularis and treatment of both lesions are reviewed. Their concurrent unrelated. (ORAL SURC ORAL MED ORAL PAIXIOL 1988;66:397-9)
D ouble lip and cheiiitis gianduiaris are both uncommon anomalies of the lips. Double lip is caused by excessive areolar tissue and noninflammatory labial mucous gland hyperplasia of the pars villosa.’ When the lip is tensed, the pars villosa sagsbelow the pars glabrosa. Cheilitis glandularis is considered to be an inflammatory hyperplasia of the lower labial salivary glands.* A unique case of concurrent double lip and cheilitis glandularis is described, and the recognition and treatment of these entities is discussed. CASE REPORT
A 65year-old white man was seen at the clinic for routine dental care. During the initial examination, bilateral redundant folds of soft tissue were noted on the mucosal surface of the upper lip. When the upper lip was tensed, the redundant tissue became evident and took the shape of a “Cupid’s bow.” The lower lip was enlarged and everted and had numerous nodular swellings. The surface was diffusely leukoplakic and dotted with several dilated secretory ducts. A thick mucoid secretion could be expressed as a result of manipulation. The patient indicated that the lower lip had occasionally become painful and clear fluid had been present overlying the inflammed secretory ducts. The lower lip was somewhat inelastic and *Associate Professor, Department of Pathology, Diagnosis, and Radiology, University of Nebraska Medical Center College of Dentistry. **Assistant Professor and Director, General Practice Residency, Department of Pathology, Diagnosis, and Radiology, University of Nebraska Medical Center College of Dentistry. ***In Private Practice, Conifer, Colo.
and
involving the minor salivary glands of the lip. A is presented. The development, diagnosis, development in this patient appears to be
The patient was fair in complexion and had a long history of exposure to the sun. He had spent considerable time in the U.S. Navy and had farmed for the past 20 years. He had been aware of his upper double lip since childhood and could not recall any other family member with a similar condition. The lesion on the lower lip had been present for several years, and he first noticed it after several severe episodes of sunburned lips. He denied having any infection and could not recall any family member who was similarly affected. Surgical treatment was recommended for both lip lesions. Even though the patient was inform4 of the high risk of squamous cell carcinoma associated with cheilitis glandularis, he remained unconcerned about the lip swellings and thought that he was too old to be worried about cosmetics. He agreed to wear a hat and use sunscreen to protect his lips when working outside. The patient’s course has been followed for 8 years, and he has had no further clinical changes in the lower lip. DISCUSSION
Double lip is a congenital or acquired oral anomaly with no known gender predilection. The congenital form is thought to develop from persistence of the horizontal sulcus between the pars glabrosa and the pars villosa during the development of the lip.3 Acquired forms, which develop later in life, have been described as occurring after trauma or in association with Ascher’s syndrome (blepharochalasis and double lip with or without nontoxic goiter).‘*4 Clinically, double lip consists of a fold of excess or 397
398 Cohen, Green, and Diekmann
Oral Surg September19X8
Fig. 1. Clinical photograph demonstrating upper double lip with lip tensed and cheilitis glandularis of lower lip. The lower lip is enlarged and everted and there are several dilated secretory ducts visible on the mucosal surface.
redundant
tissue on the mucosal side of the lip.
Although it most often occurs bilaterally on the upper lip, it may be unilateral and can affect either or both of the lips simultaneously.4-8 While the condition is not evident with the upper lip at rest, the excess or redundant tissue will project beyond the vermilion border of the lip and take the form of a “Cupid’s bow” when the upper lip is tensed.6On the lower lip, unilateral or bilateral bulbous masseswill project intraorally.6 The condition is usually present at birth but does not become apparent until the permanent teeth erupt.’ Treatment of double lip is surgical and becomes necessary for cosmetic reasons or if it interferes with speechor mastication. 6*lo The surgical procedure can be done on an outpatient basis with the use of bilateral infraorbital blocks for the upper lip and mental blocks for the lower lip. These are the preferred methods of administering anesthesia since they avoid distortion of the surgical area by infiltration.” Acquired double lip has been reported to recur after surgery, but this has not been observed with the congenital form.‘* Cheilitis glandularis is a disease of the lower lip characterized by hyperplasia of the minor salivary
glands with varying degrees of inflammation. The condition occurs primarily in adult men, but cases have been reported in women and children.‘3~‘4The exact etiology of cheilitis glandularis is unknown, although congenital predisposition and familial inheritance,13*I5 emotional disturbances,14 bacterial infection,* poor oral hygiene,16and chronic irritation from wind, sun, chemicals, and tobacco” have all been advanced as causes. Cheilitis glandularis simplex is the most common of the three clinical forms and develops as a nodular enlargement that produces eversion of the lip.13The secretory ducts become dilated and inflammed, appearing as small red or white macules on the mucosal surface.‘8 A viscid mucous secretion may ooze from them or be expressed on palpation. The superficial type (Baelz’s disease) and deep suppurative type (cheilitis glandularis apostematosa,or myxadenitis, labialis) represent progressive stages of bacterial involvement, inflammation, and enlargement of the lip. I* Eventually, deep ulceration with abscess formation and fistulous tracts may develand the lip may become two to three times op, ‘3*18*19 its normal size.‘) Scarring, secondary to persistent inffammation’and infection, causesthe Iip to become
Cheilitis
Volume 66 Number 3
inelastic.” Although the condition was originally described as painless, pain may be a variable feature of all three types and is thought to be caused by bacterial sialadenitis and mucositis.18 Cheilitis glandularis has been associated with an increased risk for the development of squamous cell carcinoma. Dysplasia has been consistently found in most casesof cheilitis glandularis, and squamous cell carcinoma has been reported to develop in 18% to 35% of the cases.Za*21 Hyperplasia of the labial salivary glands causes eversion of the lower lip, exposing the thinner, more susceptible inner labial mucosa to actinic radiation.20 Surgery is the preferred treatment for cheilitis glandularis because of the risk of dysplasia and squamous cell carcinoma.**I83** Vermilionectomy with or without lip reduction has provided satisfactory results. Antibiotics, radiation therapy, and corticosteroids have not produced consistent or sustained relief.18 SUMMARY A unique case of concurrent upper double lip and cheilitis glandularis of the lower lip has been presented, and the factors involved in the development, pathogenesis, diagnosis, and treatment of these uncommon lesions have been reviewed. Surgery is the recommended treatment modality, particularly for cheilitis glandularis because of the inherent increased risk of malignancy. The concurrent double lip and cheilitis glandularis in this patient are most likely unrelated. REFERENCES 1. Gorlin RJ, Pindborg JJ, Cohen MM. Syndromes of the head and neck. New York: McGraw-Hill, 1976:253-S. 2. Doku HC, Shklar G, McCarthy PL. Cheilitis glandularis. ORAL SURG ORAL MED ORAL PATHOL 1965;20:563-7 1. 3. Warbick JG, McIntyre JR, Ferguson AG. Remarks on the aetiology of congenital bilateral fistulae of the lower lip. Br J Plast Surg 1952;4:254-62. 4. Barnett ML, Bosshardt LL, Morgan AF. Double lip and
glandularis
and double lip
399
double lip with blepharochaiasis
(Ascher’s syndrome). ORAL
SURC ORAL MED ORAL PATHOL
1972;34:727-33.
5. Stein R. Blepharochalasis des Unterlides. Klin Monatsbl f Augenh 1930;84:846-5 1. 6. Hausamen JE, Solbach HG, Pape HD. Klinischer Beitrag zum Ascher-Syndrom. Dtsch Zahnaerztl Z 1969;24:983-7. 7. Delaire J, Landais H, Billet J, Lajarte AY. Les doubles l&es. Actual Odontostomat 1969;23:365-80. 8. Dingman RO, Billman H. Double lip. J Oral Surg 1947; 5: 146-48. 9. Converse JM. Plastic and reconstructive surgery. 2nd ed. Philadelphia: WB Saunders, 1977:1543. 10. Oldfield MC. Prolapse of the lip mucosa. Br J Surg 1959;47:58-60. . II. Schwimmer A, Dymk H, Barr C. Surgical repair of a double lin. J Am Dent Assoc 1979;99:993-4. 12. Rintila AE. Congenital double lip and Ascher syndrome. II. Relationship to the lower lip sinus syndrome. Br J Plast Surg 1981;34:31-4. 13. Weir TW, Johnson WC. Cheilitis glandularis. Arch Dermatol 1971;103:433-7. 14. Woodburne AR, Philpott OS. Cheilitis glandularis: a manifestation of emotional disturbance. Arch Dermatol Syph 1950;62:820-8. 15. Sutton RL. Cheilitis glandularis apostematosa. J Cutan Dis 1909;27: 150-5. 16. Volkmann R. Einize Falle von Cheilitis glandularis apostematosa (Myxadenitis labialis). Arch Path01 Anat 1870; 50: 142-4. 17 I I. Eve:ett FG, Ho!der T?. Chei!itis glandularis apostematosa. ORAL SURC ORAL MED ORAL PATHOL 1955;8:405-13. ID, Pickett AB. Cheilitis glandularis. ORAL ORAL MED ORAL PATHOL 1980;49:526-9.
18. Oliver
SURG
19. Shafer WG, Hine MK, Levy BM. A textbook of oral pathology. 3rd ed. Philadelphia: WB Saunders, 1974:17-8. R. Cheilitis nlandularis. Heterotonic salivary 20. Michalowski glands and squamous cell carcinoma of the lip. Br’J Dermatol 1962;74:445-9. 21. Touraine A. Les cheilites glandulaires et leur cancer. Presse Med 1950;58:1369-70. 22. Brit BD. The “lip shave” operation for premalignant conditions and microinvasive carcinoma of the lower lip. J Otolaryngol 1977;6:407- I 1. Reprint requests IO: James G. Green, D.D.S. Assistant Professor and Director Department of Pathology, Diagnosis and Radiology University of Nebraska Medical Center College of Dentistry 40th and Holdrege Lincoln, NE 68583-0740