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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 9  |  Issue : 2  |  Page : 89-92

Cheilitis glandularis of upper and lower lip in young female: Case treated with systemic steroids with follow-up of 1 year


1 Department of Oral Medicine and Radiology, SGT University, Gurugram, Haryana, India
2 Department of Prosthodontics Crown and Bridge, Rajiv Gandhi University of Health Sciences, Bengaluru, Karnataka, India

Date of Submission15-Apr-2019
Date of Acceptance11-Jul-2019
Date of Web Publication13-Nov-2019

Correspondence Address:
Dr. Jyoti Prajapat
Plot No. 39, Ranaji Enclave, Near Arya Samaj Mandir, Opp. State Bank of India, Nangli Sakrawati, Delhi - 110 043
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijohs.ijohs_19_19

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  Abstract 


There are a number of pathological conditions that may affect the lips. Cheilitis glandularis (CG) is a rare disorder characterized by hyperplasia of the labial mucous glands and mixed salivary glands as well as by ectasia of salivary glands ducts. It is a rare condition and most commonly involves the lower lip of adult males with little predilection for females. The exact etiology of this condition is unknown, but various agents have been linked. In this paper, we report a rare case of CG of the superficial suppurative type in a 20-year-old Indian female affecting upper and lower lip which was diagnosed based on clinical and histopathological findings and was treated with steroids. The present case is different because of its occurrence in a young aged female with the involvement of both lips. Early diagnosis and prompt treatment and frequent monitoring and follow-up are necessary to prevent further complications.

Keywords: Cheilitis glandularis, inflammatory disorders of lip, minor salivary glands


How to cite this article:
Prajapat J, Suma G N, Prajapat R. Cheilitis glandularis of upper and lower lip in young female: Case treated with systemic steroids with follow-up of 1 year. Int J Oral Health Sci 2019;9:89-92

How to cite this URL:
Prajapat J, Suma G N, Prajapat R. Cheilitis glandularis of upper and lower lip in young female: Case treated with systemic steroids with follow-up of 1 year. Int J Oral Health Sci [serial online] 2019 [cited 2019 Dec 15];9:89-92. Available from: http://www.ijohsjournal.org/text.asp?2019/9/2/89/270876




  Introduction Top


Cheilitis glandularis (CG) is a rare chronic inflammatory disease that most commonly affects the minor salivary glands of lips and is characterized clinically by edema and focal ulceration. The term CG was first used by Volkmann in 1870 to describe a disorder that is characterized by chronic, suppurative inflammation of the lower lip, with swelling of the mucous glands and mucopurulent discharge through dilated ductal openings.[1] This was later reported under various terminologies such as CG simplex, CG superficialis, and CG apostematosa.[2] CG on long term can progress to CG apostematosa – a severe deep-seated inflammation characterized by the formation of abscesses and fistulous tracts.[3]

Based on clinical presentation, glandular cheilitis can be classified into simplex (described by Puente and Acevedo), superficial suppurative (described by Baelz-Unna), and a more severe deep suppurative type, also known as myxadenitis labialis or CG apostematosa (Volkmann's cheilitis), characterized by deep-seated inflammation forming abscesses and fistulous tracts.[4]

CG most commonly involves lower lip of an adult male with little predilection to female and children; it mostly affects middle-aged to older Caucasian men; Japanese and Asian cases are very rare.[1] Although there is male predilection, cases have been reported in women and children also. Familial cases have also been reported in literature.[2]

It is associated with multiple etiological factors such as syphilis, bacterial infections, actinic radiation, tobacco, poor oral hygiene, and genetic transmission. Although this disorder is related to chronic ultraviolet exposure, it is not precancerous but is a marker of comorbid malignancy and/or significant disease of the oral cavity.[5]

Clinically, CG may resemble many other conditions. Differential diagnoses include multiple mucocele, chronic sialadenitis of the minor salivary glands, sialolithiasis of minor salivary gland, factitious cheilitis, actinic cheilitis, cheilitis granulomatosa, angioedema, and benign and malignant salivary gland tumors.[1]


  Case Report Top


A 20-year-old female patient reported to our department with a chief complaint of swelling of her upper and lower lip with crusting [Figure 1] and occasional discharge of fluid for 1 year. According to the patient, swelling of lips started 1 year back with symptoms of dryness and redness of both lips which later was associated with bleeding from the dry areas upon stretching and formation of crust with occasional mucopurulent discharge. There is frequent alteration in size of the swelling. The patient gives a history of working in fields. There was no family history with the same condition.
Figure 1: Profile picture of the patient with biopsy site

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On inspection, the upper and lower lip was diffusely swollen and everted, dry, incompetent with a well-defined solitary ulcerated lesion above vermillion border of size 3 cm × 1 cm with crustaceans on the lower lip [Figure 2]. There was loss of lip contour. Swelling with few diffuse flecks of crust was also seen on the upper lip vermillion border [Figure 3]. Multiple superficial erosions and crust were seen, but no mucopurulent discharge was seen at the time of examination. Lower lip was inflamed and swollen. On palpation, the lip was slightly tender, edematous, and soft in consistency and indurated. Patient had poor oral hygiene.
Figure 2: Everted lower lip with crustation

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Figure 3: Upper lip with crustation

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On the basis of above clinical findings, it was provisionally diagnosed as CG. Differential diagnosis given was actinic cheilitis, atopic dermatitis, cheilitis granulomatosa, and syphilitic ulcer. Routine blood investigations were within normal limits. Incisional biopsy on the lower lip was done to confirm the diagnosis. Hematoxylin and eosin-stained section shows minor salivary gland tissue with chronic inflammatory cell infiltration and ductal dilatation. Connective tissue stroma shows collagen fibers with numerous engorged blood vessels, fibroblasts, and numerous mucous salivary acini. The glandular tissue was showing degenerative changes. Inflammatory cell infiltration was seen throughout the connective tissue stroma. Overlying epithelium was proliferative showing acanthosis. Histopathological report was suggestive of CG [Figure 4]. Hence, a final diagnosis of CG was made on the basis of clinical features and histopathology report. The patient was treated with systemic prednisone 10 mg three times a day for 7 days. The patient was also referred to the department of periodontics for oral prophylaxis. After 7 days, the dose of prednisone was reduced to 10 mg two times a day for the next 5 days. On the next follow-up, the patient dose of prednisone was further reduced to 10 mg once daily for the next 5 days. The patient was advised to stop medication and recall for follow-up [Figure 5] and [Figure 6]. The present case is under follow-up for 1 year with all local measures in controlling the recurrence.
Figure 4: Photomicrograph showing minor salivary gland tissue with chronic inflammatory cell infiltration and ductal dilatation

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Figure 5: After follow-up of 3 months

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Figure 6: After follow-up of 6 months

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  Discussion Top


CG is an uncommon, rare inflammatory disorder of the lower lip. Sutton (1914) stated that the characteristic lip swelling is due to a congenital adenomatous enlargement of the labial salivary glands.[6]

In the German-speaking literature, an affection of the lower lip termed “acute ectropionizing bacterial cheilitis” has been described which marks a differential diagnosis of CG. According to Bork et al. (1993), this uncommon disease is caused by exacerbation of folliculitis or furuncle affecting the adjacent skin. Acute ectropionizing bacterial cheilitis begins with edema of the lower lip. Then, within a few hours, inflammatory infiltration, eversion, and crusts develop on the mucosal part of the lip. In contrast to CG, abscess-forming or phlegmonous inflammation is not observed.[3]

The exact etiology of CG is unknown, but some authors say that it is a hereditary autosomal dominant condition.[7]

CG occurs more in adult males; however, cases have been rarely reported in women and in children with wide age ranging between fourth and seventh decades of life. According to English literature only few cases (12 cases) in teenagers and young adults[8],[9],[10] have been reported with possible predisposing factors such as poor oral hygiene, chronic sun exposure, and a compromised immune status.

The present case is different because of its occurrence in a young-aged female with the involvement of both lips and possible etiological role of exposure to sunlight.

In literature, clinical variants of CG include simple, superficial suppurative, and deep suppurative CG. Simple CG is characterized by thickening of only lower lip; on eversion of lower lip, it exposes widely dilated, sieve-like openings scattered irregularly along the vermilion border and exuding a glistening mucoid secretion. These accessory minor salivary glands can be palpated, are enlarged and tender if suppurative, and may accept a small probe through their dilated and ectatic ductal openings, which are clearly visible as bright red papules scattered over the surface of the lip. Patient complaints include sticky or tacky lips and suppurative abscesses if occlusive creams or ointments inadvertently introduce bacteria or yeast into the ductal structures.[11]

Gileva et al. studied the frequency and spectrum of oral manifestations of HIV-infected drug-users in the Perm region and found purulent form of CG in 3.9% of HIV-infected drug abusers.[12]

The superficial suppurative type (Baelz disease) consists of painless, indurated swelling of the lip with shallow ulceration and crusting. CG of deep suppurative type comprises a deep-seated infection with the formation of abscesses, sinus tracts, and fistulas and potential for scarring. The latter two types have the highest association with dysplasia and carcinoma.[13]

Microscopy shows variably dilated and tortuous minor salivary gland ducts, together with interstitial acute and chronic inflammation. Many of the ducts are lined by oncocytic cells with foci of hyperplasia, and there may be intraluminal papillary projections. Mucous and squamous metaplasia may be present. The surrounding minor glands show acinar atrophy, interstitial fibrosis, and inflammation. In addition, there may be interstitial mucous extravasation and occasionally epithelioid granuloma formation.[14]

The differential diagnosis of CG includes contact cheilitis attributed to irritants or allergic contactants such as cheilitis medicamentosa; plasma cell cheilitis, exfoliative cheilitis related to factitial processes such as lip licking; and cheilitis granulomatosa affecting the upper and lower lip, and characterized by tuberculoid granulomatous inflammation, lingua plicata, and peripheral seventh nerve palsy (Melkersson–Rosenthal syndrome).[11]

Different treatment modalities have been reported for CG and vary accordingly depending upon the predisposing factors, including antibiotics, antihistamines, steroids, immunosuppressants, radiotherapy, surgical stripping, vermilionectomy, cryosurgery, or laser surgery or topical chemotherapy with 5-fluorouracil. Recurrence is rare after surgery.[1]


  Conclusion Top


We suggest that CG should be considered in differential diagnosis of lip swelling and also the patient with CG should be monitored carefully, because on long term some patients may be at risk for the development of suppurative episodes because frequent trauma to the lip surface forms portals of entry for bacterial invasion, and few cases also have the potential for the development of lip carcinoma. In CG, for cases with a history of chronic sun exposure, biopsy is recommended to rule out actinic cheilitis or carcinoma.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Mohammed RB, Rani GS, Sandya MP, Jyotsna S. Cheilitis glandularis treated with intralesional steroids: A rare case report and review. Oral Maxillofac Pathol J 2014;5:510-3.  Back to cited text no. 1
    
2.
Bhat S, Shetty SR, Majumdar PK, Bhat S, Fazil KA. Cheilitis glandularis – Review of a rare inflammatory disorder. Balkan Military Medical Review 2016;19:25-28.  Back to cited text no. 2
    
3.
Hillen U, Franckson T, Goos M. Cheilitis glandularis: A case report. Acta Derm Venereol 2004;84:77-9.  Back to cited text no. 3
    
4.
Paravina M. Cheilitis glandularis apostematosa in a female patient – A case report. Serbian J Dermatol Venereol 2013;5:177-82.  Back to cited text no. 4
    
5.
Wazir SS, Srivastava R, Arora P. Chelitis Glandularis of the Lower Lip-A Case Report. Int J Oral Health Adv 2013;1(1):5-9.  Back to cited text no. 5
    
6.
Neville BW, Damm DD, Allen CA, Bouquot JE. Salivary gland pathology. In: Oral & Maxillofacial Pathology. 3rd ed. St Louis, Mo: Saunders Elsevier; 2009. p. 462-3.  Back to cited text no. 6
    
7.
Dhanapal R, Nalin Kumar S, Saraswathi TR, Uma Devi M, Joshua E, Veerabahu M, et al. Maxillary double lip & cheilitis glandularis: Unusual occurrence. J Oral Maxillofac Pathol 2007;11:35-7.  Back to cited text no. 7
  [Full text]  
8.
Nesbitt RA, Marley JJ, James J. Cheilitis glandularis: Report of an unusual case affecting the upper lip. Oral Surg 2010;3:32-5.  Back to cited text no. 8
    
9.
Ellen Eisenberg, Dirk M., Elaston. Cheilitis Glandularis Follow up; http://www.emedicine.medspace.com/article/1078725; [Last accessed on 2019 Aug 22].  Back to cited text no. 9
    
10.
Yacobi R, Brown DA. Cheilitis glandularis: A pediatric case report. J Am Det Assoc 1989;118:317-8.  Back to cited text no. 10
    
11.
Carrington PR, Horn TD. Cheilitis glandularis: A clinical marker for both malignancy and/or severe inflammatory disease of the oral cavity. J Am Acad Dermatol 2006;54:336-7.  Back to cited text no. 11
    
12.
Gileva OS, Sazhina MV, Gileva ES, Efimov AV, Scully C. Spectrum of oral manifestations of HIV/AIDS in the perm region (Russia) and identification of self-induced ulceronecrotic lingual lesions. Med Oral 2004;9:212-5.  Back to cited text no. 12
    
13.
Sreedhar G, Sumalatha MN. Cheilitis glandularis: A case report. Int J Oral Maxillofac Pathol 2011;2:24-7.  Back to cited text no. 13
    
14.
Eveson JW, Speight PM. Non – Neoplastic lesions of the salivary glands: New entities and diagnostic problems. Curr Diagn Pathol 2006;12:22-30.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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