Marcia Ramos-e-Silva

Associate Professor and Supervisor of the Oral Dermatology Out-Patient Clinic
Sector of Dermatology - Department of Medical Clinics
From the Post-Graduation Course in Dermatology, Hospital Universitário Clementino Fraga Filho, School of Medicine, Universidade Federal do Rio de Janeiro, Brazil.

Abstract:The author presents a case of Volkmann's cheilitis that developed a squamous cell carcinoma. Although radiotherapy is not the treatment of choice for well-differentiated squamous-cell carcinoma, it showed good results in the lesion's size reduction, and a less aggressive surgery could then be performed to eradicate the neoplasm.

Key words: Carcinoma, squamous cell; Cheilitis; Lip neoplasms; Radiotherapy

INTRODUCTION
Volkmann's cheilitis is a rare entity associated with enlargement of the salivary glands of the lower lip, intense mucous discharge and frequent secondary infections. Enlargement becomes permanent specially after multiple infections and malignancy develops in approximately 20% of the cases. There are very few papers about this disease in the World Literature and a case of malignant change in this rare type of cheilitis with good esthetical and therapeutical results after radiotherapy and surgery is presented.

CASE REPORT
A 40-years-old white-male, from the city of Rio de Janeiro, presented, for the past 4 years, papules, nodules and multiple fistules with mucous discharge, affecting mostly the left lower lip. Its growth was very slow, but during the last year it was faster. The lesion became infiltrated and spread to the adjacent gingiva. There was no cervical or submandibular adenomegaly and a purulent discharge from the lesion was observed. (Figure 1, 2)

The main laboratorial exams showed infection by Staphylococcus aureus; absence of fungi; negative exams for syphilis, HIV, and hepatitis; negative Montenegro intradermal test for leishmaniasis; and histopathology of the lesion showed a well-differentiated squamous-cell carcinoma, a neoplasm composed by groups of squamous cells and dyskeratosis.

Before coming to our hospital he was treated with topical applications of 5-fluorouracil without improvement. Our choice was to perform radiotherapy for reduction of size and posterior surgery. There was an overall good esthetical result even before surgery. (Figure 3) The patient will be in observation every three months for detection of any relapse of the tumor.

DISCUSSION
Volkmann's cheilitis, apostematous cheilitis or cheilitis glandularis is a rare chronic condition of unknown etiology that produces a great, permanent and usually symmetrical enlargement of the labial mucous and salivary glands of the lips, specially the lower.1,2,3,4,5

This type of macrocheilitis is known since 1687, when it was observed by Cornelius Stalpart Van Der Wiel, but it was fully described by Richard von Volkmann, who published a paper with five personal observations of what he called cheilitis glandularis apostematosa or myxadenitis labialis.1,3

It can begin during childhood, adolescence 3 or adulthood,5 occurs mainly in whites, and is twice as frequent in males than in females.3,5 Some authors associate this condition with smoking, emotional distress, poor oral hygiene, chronic sun and wind exposure, bacterial infection and congenital factors.2,4,5

Three forms of cheilitis glandularis may be described: a simple and most frequent form, which has not yet secondary infection, first mentioned by Puentes and Acevedo, in 1927, but already suggested by Sutton, in 1914;6 and two suppurative forms, the superficial, called the Baelz-Unna type, and the deep suppurative form, which corrresponds to the one studied by Volkmann,1 with more severe involvement of the lower lip occurring after several bacterial infections and showing intense mucous secretion.3,4

In this more severe form, called Volkmann cheilitis, the lower lip is atrophic, dry, fissured, scaly, usually everted, and, from the openings of the enlarged salivary glands ducts, there is a viscous, yellowish, mucopurulent discharge Multiple secondary infection of the deep labial fissures results in fistulization, crusting, abscesses and scarring. There are inflammatory changes, great enlargement of the lower lip, intense mucous discharge. Malignization into a squamous-cell carcinoma occurs in 20% of the cases.5

It is not a true cheilitis but was postulated to be a hypertrophy of the salivary glands of the lips, also showing heterectopic glands, and resulting in secretion retention, pseudocystic dilatation and nodule formation. The main diagnostic sign is the mucous discharge when the affected area is compressed.3 Swerlick & Cooper, in 1984, after a thorough microscopic examination of their five personal cases and analysis of microphotographs of 48 cases in the literature, stated that Volkmann's cheilitis represents an unusual reaction pattern in response to chronic irritation of the lips and is unrelated to labial salivary gland hyperplasia.6 Rada et al. proposed a ductal origin because there is clinically ductal ectasia and metaplasia. In their cases this histologic ductal ectasia was striking and they suggested that the discrepancy between clinical appreciation of dilated ductal orifices and that seen histologically of many cases in the literature may be due to tissue orientation during processing or to the lack of serial sectioning.7

Swerlick & Cooper divided cheilitis glandularis into three categories: the largest group develops on fair-skinned individuals, with premature skin and lip chronic sun and/or wind damage, who frequently show malignant transformation of the lesion. The second group has atopic history and the authors postulated that the constant dryness of the lips caused by mouth breathing with or without chronic sun exposure could act as irritant. In the third group, excessive or inadequate wetting, lip biting and/or other manipulations act as the irritant factor and these patients have factitious cheilitis rather than cheilitis glandularis. Histopathology does not help in differentiating the three categories.6 There are no consistent changes in the minor salivary glands to form a basis for histologic diagnosis and the lack or the presence of varying degrees of inflammation is considered a secondary change.7

Doku et al.,8 Williams & Williams,9 and Lederman10 observed patients with oral lesions very similar to cheilitis glandularis on sites other than the lips. This was called persistent sialoadenitis of the minor glands or stomatitis glandularis,9 the latter term bring preferred.10

Volkmann cheilitis must be differentiated from other lip enlargements such as cheilitis of Melkersson-Rosenthal or cheilitis granulomatosa, sarcoidosis, Crohn's disease, lymphangioma, tuberculosis, angioneurotic edema and chronic actinic cheilitis. 2,3,4

Treatment is not required for the simple cases in which emollients and sunscreens can provide some protection. Antibiotics are used for secondary infections and topical steroids may improve inflammatory changes. In more severe cases vermilionectomy can be necessary.3,4

Our patient had the deeper suppurative type of cheilitis glandularis, known as Volkmann cheilitis, was classified as type 1 of Swerlick and Cooper,6 and showed transformation to a well differentiated squamous cell carcinoma. This malignization probably began one year before, when the larger infiltration and more rapid growth occurred. There was a good therapeutical result with radiotherapy, performed to try a shrinkage of the lesion, before vermilionectomy was performed.

ACKNOWLEDGMENTS
The author thanks the collaboration of Profs. Ataliba Belizzi (Department of Surgery), Francisco Campana, Paulo Camary (Sector of Radiotherapy) and Olga Harris (Department of Pathology) and Doctors Ana Paula Costa, Claudia Câmara and Omar LR Santos (Post Graduation Course in Dermatology), in the patient's diagnostic elucidation and treatment.

REFERENCES
1. von Volkmann R. Einize falle von cheilitis glandularis apostematosa (Myxadenitis labialis). Virchows Arch 1870;50:142-4.
2. Oliver ID, Pickett AB. Cheilitis glandularis. Oral Surg Oral Med Oral Pathol 1980;49:526-9.
3. Bork K, Hoede N, Korting GW. Doenças e Sintomas da Cavidade Bucal e da Região Perioral - Atlas Colorido. São Paulo: Manole, 1988; 36-9.
4. Laskaris G. Color Atlas of Oral Diseases. Stuttgart: Thieme, 1988; 90.
5. Langlais RP, Miller CS. Color Atlas of Common Oral Diseases. Philadelphia: Lea & Febiger, 1992; 30.
6. Swerlick RA, Cooper PH. Cheilitis glandularis: a reevaluation. J Am Acad Dermatol 1984;10:466-72.
7. Rada DC, Koranda FC, Katz FS. Cheilitis glandularis: a disease of ductal ectasia. J Dermatol Surg Oncol 1985;11:372-5.
8. Doku HC, Shklar G, McCarthy PL. Cheilitis glandularis. Oral Surg Oral Med Oral Pathol 1965;20:563-71.
9. Williams HK, Williams DM. Persistent sialoadenitis of the minor glands: stomatitis glandularis. Br J Oral Maxillofac Surg 1989;27:212-6.
10. Lederman DA . Suppurative cheilitis glandularis. Oral Surg Oral Med Oral Pathol 1994;78:319-22.

Figure 1: Clinical aspect of the Volkmann's cheilitis and squamous cell carcinoma.
Figure 2: Clinical aspect showing the inner lower lip.
Figure 3: Aspect of the lesion two months after radiotherapy and before surgery.