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CASE REPORT
Year : 2017  |  Volume : 6  |  Issue : 3  |  Page : 174-177

Cheilitis granulomatosa: Case series


1 Department of DVL, Government Medical College, Nizamabad, Telangana, India
2 Department of Oral Medicine, Viswanath Katti Institute of Dental Sciences, Belgaum, Karnataka, India
3 Department of DVL, Gandhi Medical College and Hospital, Hyderabad, Telangana, India
4 Department of Pathology, Gandhi Medical College and Hospital, Hyderabad, Telangana, India

Date of Web Publication25-Sep-2017

Correspondence Address:
Sudha Rani Chintagunta
Plot No. 5, Jupiter Colony, Kakaguda, Karkhana, Secunderabad, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JDRNTRUHS.JDRNTRUHS_13_17

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  Abstract 

Granulomatous cheilitis is an idiopathic inflammatory disorder characterized by persistent, asymptomatic, diffuse swelling of one or both lips. It is also considered as an oligosymptomatic or monosymptomatic form of Melkersson–Rosenthal syndrome. We herewith report five cases of granulomatous cheilitis of which three cases were females and two were males. The mean age was 41 years. The average duration of symptoms was 2.5 years (2–3 years). Four patients presented with asymptomatic persistent swelling of the upper lip and one patient with lower lip. Two cases showed associated fissuring and angular cheilitis. One case also showed involvement of the left cheek. All the patients showed moderate improvement with combination of minocycline and intralesional triamcinolone therapy.

Keywords: Cheilitis granulomatosa, combination therapy, noncaseating granulomas


How to cite this article:
Chintagunta SR, Sana SN, Bukka KS, Padma S. Cheilitis granulomatosa: Case series. J NTR Univ Health Sci 2017;6:174-7

How to cite this URL:
Chintagunta SR, Sana SN, Bukka KS, Padma S. Cheilitis granulomatosa: Case series. J NTR Univ Health Sci [serial online] 2017 [cited 2019 Nov 22];6:174-7. Available from: http://www.jdrntruhs.org/text.asp?2017/6/3/174/215520


  Introduction Top


Granulomatous cheilitis (Miescher cheilitis) is the most common monosymptomatic form of Melkersson–Rosenthal syndrome (MRS), characterized by recurrent swelling of the lips.[1] We report four patients who presented with persistent asymptomatic swelling of the upper lip and lower lip in one patient. One patient also had swelling in the perioral region and cheek. One of the female patients had a family history of persistent swelling of the upper lip.


  Case Reports Top


Case 1

A 58-year-old female presented with asymptomatic persistent swelling of the upper lip and erythematous patch on the left cheek for 3 years. Cutaneous examination revealed diffuse thickening of the upper lip and well-defined erythematous, nontender, indurated plaque on the left mandibular region [Figure 1]. There was no sensory loss over the plaque. Intraoral examination and systemic examination revealed no abnormalities.
Figure 1: Swelling of the upper lip and erythematous plaque on the left cheek

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Case 2 and 3

A 23-year-old male and 58-year-old male presented with a 2-year history of asymptomatic swelling of the upper lip and lower lip, respectively. Cutaneous examination revealed diffuse swelling of the upper lip, firm in consistency, and nontender. Angular cheilitis was present. Intraoral and systemic examination did not reveal any abnormality [Figure 2] and [Figure 3].
Figure 2: Swelling of the upper lip with fissuring and scaling

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Figure 3: Upper lip swelling and angular cheilitis

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Case 4

A 38-year-old female presented with asymptomatic persistent swelling of the upper lip for 3 years. Cutaneous examination revealed diffuse swelling of the upper lip with fissuring and scaling, firm in consistency, and nontender. Intraoral examination revealed no abnormalities [Figure 4].
Figure 4: Swelling of the lower lip

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Case 5

A 34-year-old female presented with persistent asymptomatic swelling of the both lips and erythematous granular appearance of the gums for 2 years.

A differential diagnosis of granulomatous cheilitis and borderline tuberculoid Hansen's disease was considered in case one [Figure 1]. Glandular cheilitis, granulomatous cheilitis, and sarcoidosis were considered in the other four cases. We did biopsy in four cases and all the cases showed noncaseating granulomas. The fifth one came with report suggestive of granulomatous cheilitis.

All the routine investigations and chest X-ray were normal. In case, one slit skin smear taken from the plaque on the left cheek was negative for acid-fast bacilli. Skin biopsy on H and E section studied shows scanner view of skin with epidermis showing keratinized stratified squamous epithelium along with dermis showing adenexae and chronic inflammatory infiltrate composed of lymphocytes, plasma cells, histiocytes, and occasional multinucleated giant cells [Figure 5]. High-power view revealed noncaseating granuloma composed of lymphocytes, plasma cells, histiocytes, epithelioid cells, and multinucleated giant cells [Figure 6].
Figure 5: Normal epidermis and dermis showing adnexa and chronic inflammatory infiltrate composed of lymphocytes, plasma cells, histiocytes, and occasional multinucleated giant cells, H and E, x10

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Figure 6: Noncaseating granuloma composed of lymphocytes, plasma cells, histiocytes, epithelioid cells, and multinucleated giant cells, H and E, ×40

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Based on the clinical features and histopathology, a diagnosis of cheilitis granulomatosa (CG) was made. Four patients were started on minocycline 100 mg daily and intralesional triamcinolone 10 mg/ml every 2 weeks. After 6 weeks, there was moderate improvement in the labial swelling, and minocycline was continued on alternate days for another 8 weeks. One patient was on oral prednisolone before attending this institute, and we tapered the steroid dose along with addition of minocycline 100 mg daily. Three cases lost to follow-up (two patients are from other country). Two patients are under follow-up, showing response whenever on medication, relapse after stopping the treatment in one and another patient is in remission for the past 6 months.


  Discussion Top


Orofacial granulomatosis is characterized by granulomatous inflammation affecting the soft tissues of the oral and maxillofacial region and includes MRS and CG of Miescher. MRS is a triad of persistent lip swelling, facial nerve paralysis, and fissured tongue[2],[3] The complete triad is present in 8%–25% of patients, and oligosymptomatic forms of the condition account for 47% of cases. Granulomatous cheilitis is the sole manifestation of the disease in 28% of cases.

Granulomatous cheilitis is a monosymptomatic form or an incomplete variant of MRS.[3] Usually, it affects young adults with a female predilection. The estimated incidence of CG is 0.08% in the population. The various etiological factors proposed are genetic predisposition, allergic reactions, chronic dental infections, autoimmune, association with Crohn's disease and sarcoidosis, and sensitization to metals such as gold and mercury. Various substances such as chocolate, food additives, cinnamon compounds, and benzoates have also been implicated as possible etiologic agents.

The most common presentation is persistent asymptomatic swelling of the upper or lower or both lips.[1] The swelling is soft to firm in consistency and nontender and may associated with erythema, erosions, fissuring, or scaling of the lips. The disease can also affect the oral and other facial regions, including the chin, cheeks, periorbital region, and eyelids.[4] Oral cavity involvement includes erythema of oral mucosa, gums, tongue, pharynx, and larynx.[5] The clinical course and therapeutic response are often unpredictable with spontaneous remissions and relapse.

The clinical presentation of CG is highly variable. Literature shows that upper lip involvement was the most common presentation. The degree of lip swelling also varied and is frequently associated with both vertical fissuring and angular cheilitis.

In our series, we have observed five cases of granulomatous cheilitis and appear to be idiopathic in origin. Four patients showed persistent swelling of the upper lip and lower lip involvement in one. The first case presented with erythematous plaque of longer duration on the left cheek mimicking other granulomatous conditions along with lip hypertrophy [Figure 1]. Two patients had scaling, fissuring, and perioral erythema mimicking factitial cheilitis [Figure 3] and [Figure 4] and one with angular cheilitis [Figure 2]. Although the clinical presentation is variable, in all the five cases, skin biopsy was consistent with granulomatous cheilitis. All the patients showed moderate improvement with combination treatment with minocycline and intralesional triamcinolone.

Various treatment modalities are in use without satisfactory outcome. First-line treatment is local or systemic corticosteroids or both. Other therapeutic measures include hydroxychloroquine, methotrexate, clofazimine, metronidazole, minocycline alone, or in combination with oral prednisone, thalidomide, dapsone, and danazol. Combination therapy with intralesional triamcinolone and clofazimine or dapsone is one of the most commonly used treatment options.[6],[7] Various reports showed successful results with combination of intralesional steroids and metronidazole[8] and combination of intralesional triamcinolone, metronidazole, and minocycline.[9],[10],[11] Cheiloplasty is useful in patients with severe disfigurement of the lips.


  Conclusion Top


The clinical presentation of granulomatous cheilitis is variable, and the diagnosis is by exclusion of other granulomatous lesions which produce the similar clinical and histopathological features. Awareness about this condition, appropriate clinical evaluation, and skin biopsy helps in the early diagnosis and management.

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.
El-Hakim M, Chauvin P. Orofacial granulomatosis presenting as persistent lip swelling: Review of 6 new cases. J Oral Maxillofac Surg 2004;62:1114-7.  Back to cited text no. 1
    
2.
Greene RM, Rogers RS 3rd. Melkersson-rosenthal syndrome: A review of 36 patients. J Am Acad Dermatol 1989;21:1263-70.  Back to cited text no. 2
    
3.
Spielmann AC, Maury F, George JL. Melkersson-rosenthal syndrome: Anatomo-clinical and evolutive concepts. J Fr Ophtalmol 2000;23:261-4.  Back to cited text no. 3
    
4.
Mignogna MD, Fedele S, Lo Russo L, Lo Muzio L. The multiform and variable patterns of onset of orofacial granulomatosis. J Oral Pathol Med 2003;32:200-5.  Back to cited text no. 4
    
5.
van der Waal RI, Schulten EA, van de Scheur MR, Wauters IM, Starink TM, van der Waal I, et al. Cheilitis granulomatosa. J Eur Acad Dermatol Venereol 2001;15:519-23.  Back to cited text no. 5
    
6.
Sobjanek M, Michajłowski I, Zelazny I, Medrzycka-Dabrowska W, Włodarkiewicz A. What is the most effective treatment of cheilitis granulomatosa in Melkersson-rosenthal syndrome? J Eur Acad Dermatol Venereol 2010;24:364-5.  Back to cited text no. 6
    
7.
Sobjanek M, Włodarkiewicz A, Zelazny I, Nowicki R, Michajłowski I, Sokołowska-Wojdyło M, et al. Successful treatment of Melkersson-rosenthal syndrome with dapsone and triamcinolone injections. J Eur Acad Dermatol Venereol 2008;22:1028-9.  Back to cited text no. 7
    
8.
Coskun B, Saral Y, Cicek D, Akpolat N. Treatment and follow-up of persistent granulomatous cheilitis with intralesional steroid and metronidazole. J Dermatolog Treat 2004;15:333-5.  Back to cited text no. 8
    
9.
Dar NR, Raza N, Nadeem A, Manzoor A. Granulomatous cheilitis: Sustained response to combination of intralesional steroids, metronidazole and minocycline. J Coll Physicians Surg Pak 2007;17:566-7.  Back to cited text no. 9
    
10.
Martínez Martínez ML, Azaña-Defez JM, Pérez-García LJ, López-Villaescusa MT, Rodríguez Vázquez M, Faura Berruga C, et al. Granulomatous cheilitis: A report of 6 cases and a review of the literature. Actas Dermosifiliogr 2012;103:718-24.  Back to cited text no. 10
    
11.
Gupta A, Singh H. Granulomatous cheilitis: Successful treatment of two recalcitrant cases with combination drug therapy. Case Rep Dermatol Med 2014;2014:509262.  Back to cited text no. 11
    


    Figures

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



 

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