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Table of Contents 
E–CASE REPORT
Year : 2013  |  Volume : 58  |  Issue : 3  |  Page : 242
Cheilitis granulomatosa: A case report with review of literature


1 Department of Oral Pathology and Microbiology, School of Dental Sciences, Krishna Institute of Medical Sciences, Deemed University, Karad, India
2 Department of Orthodontia, Bharati Vidyapeeth Dental College and Hospital, Pune, India
3 Department of Periodontology, Saraswati Dhanwantari Dental College and Hospital, Parbhani, Maharashtra, India

Date of Web Publication20-Apr-2013

Correspondence Address:
Nupura A Vibhute
C/O Dr. HG Vibhute, Opposite Hotel Greenfield, Uttekaranagar, Sadar Bazar, Satara, Maharashtra - 415 001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.110858

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   Abstract 

Cheilitis granulomatosa (CG) is a chronic swelling of the lip due to granulomatous inflammation. It is a rare inflammatory disorder first described by Miescher in 1945. It is a monosymptomatic form or an incomplete variant of Melkersson-Rosenthal syndrome; a triad of recurrent orofacial edema, recurrent facial nerve palsy and fissuring of the tongue. As the etiology remains unknown, treatment of CG is challenging. We present a case of CG in a 43-year-old female patient secondary to allergy to certain food items. The present case highlights the importance of thorough investigations in the diagnosis of this lesion as the findings mimic many other granulomatous conditions.


Keywords: Cheilitis granulomatosa, lip swelling, Melkersson-Rosenthal syndrome


How to cite this article:
Vibhute NA, Vibhute AH, Daule NR. Cheilitis granulomatosa: A case report with review of literature. Indian J Dermatol 2013;58:242

How to cite this URL:
Vibhute NA, Vibhute AH, Daule NR. Cheilitis granulomatosa: A case report with review of literature. Indian J Dermatol [serial online] 2013 [cited 2018 Aug 15];58:242. Available from: http://www.e-ijd.org/text.asp?2013/58/3/242/110858

What was known? Cheilitis Granulomatosa is a rare inflammatory disorder with unknown etiology. This renders this granulomatous condition difficult to treat



   Introduction Top


Cheilitis granulomatosa (CG) is a rare and unique disorder. This condition is characterized by chronic swelling of one or both the lips due to granulomatous inflammation. It is a rare inflammatory disorder first described by Miescher in 1945. [1],[2] It is a monosymptomatic form or an incomplete variant of Melkersson-Rosenthal syndrome (MRS); a triad of recurrent orofacial edema, recurrent facial nerve palsy and fissuring of the tongue. CG is also considered a subset of an uncommon disease Orofacial granulomatosis, which was introduced by Wiesenfeld in 1985. [3]


   Case Report Top


A 43-year-old woman patient presented with persistent, painful upper lip swelling since one week [Figure 1]. There was no history of applied irritants, local trauma or atopy. She also reported burning and peeling off in perioral area. Past history revealed that patient had been treated for similar condition with various different modalities including ayurvedic medication, homeopathy and topical steroids in past three years. On examination the upper lip and perioral area showed painful swelling, with areas of crusting and fissuring [Figure 2]. Neither facial nerve palsy nor fissuring of the tongue nor lymph node involvement was present. No such lesions were seen intraorally.
Figure 1: Patient with upper lip swelling and peeling in perioral area

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Figure 2: Upper lip and perioral area showing swelling and areas of crusting and fissuring

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The biopsy revealed hyperplastic epithelium of variable thickness covered by fibrinopurulent exudate [Figure 3]. The superficial lamina propria was loose and edematous. The underlying fibrocellular connective tissue revealed with abundant non caseating granulomas with Langhans type multinucleated giant cells and perivascular lymphocyte aggregations [Figure 4]. In other investigations, chest radiograph showed no abnormalities, and a Mantoux test was non-reactive. Evaluation for food allergies revealed higher allergen quantity for the following food items (normal range being 0-0.35 Ku/L):
Figure 3: Section showing hyperplastic epithelium and dense inflammatory infiltrate in underlying connective tissue (H and E, ×10)

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Figure 4: Section under higher magnification showing fibrocellular connective tissue with abundant non - caseating granulomas with Langhans type multinucleated giant cells (H and E, ×40)

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Almond: 1.90

Cashew nut: 1.30

Urad dal: 1.30

Rajma: 1.20

Cabbage: 1.80

Tomato: 2.00

From all these findings a diagnosis of CG secondary to certain food items was made.


   Discussion Top


CG is a chronic swelling of the lip due to granulomatous inflammation. It is a monosymptomatic form or an incomplete variant of MRS which is a triad of recurrent orofacial swelling, relapsing facial paralysis and fissuring of the tongue (lingua plicata). Presentation of complete MRS with all three elements of the triad in a single patient is rare, being reported in only 10-20% of cases, and bilateral facial palsy in MRS is even rarer. [4] In approximately 40% of cases, CG is the presenting sign of MRS, with subsequent development of the neurologic signs. [4]

The presence of CG without lingua plicata or facial palsies also is called Miescher syndrome or Miescher's Cheilitis. [1],[2] In 1985, Wiesenfield [3] introduced the concept of Orofacial granulomatosis (OFG). Today both CG and MRS are considered subsets of OFG.

The etiology of CG continues to remain a mystery. Reports suggest that some cases may demonstrate an autosomal dominant inheritance pattern, with the responsible gene mapping to chromosome 9 p11. [5] Other authors have proposed a wide range of causes including allergic reactions, chronic infectious odontogenic foci, autoimmune mechanism, as an association with Crohn's disease and sarcoidosis or even as oral manifestation of systemic diseases. [6]

The age of onset is usually young adulthood with no racial or sexual predilection.

Zimmer, [7] et al. in their analyses of 42 patients and review of 220 cases they showed female predilection and a wide range of onset with a mean of 33.8 years.

Granulomatous cheilitis should be considered in children as well according to Oliver, et al.[8] who reported Granulomatous Cheilitis in an eight-year-old girl, which persisted for more than one year.

In patients presenting with CG it is important to perform an appropriate evaluation, which includes a chest radiograph and a Mantoux test to exclude other etiologies of granulomatous disease, such as sarcoidosis or Mycobacterium infection. Gastrointestinal tract endoscopy and radiography may be used to exclude Crohn's disease. Patch tests have implicated cobalt and the food additives cinnamaldehyde and benzoates in the pathogenesis of CG. [3]

Treatment

Various treatments for CG have been reported, including antibiotics like tetracycline and clofazimine [8],[9] tranilast, [10] oral and intralesional steroids, [11],[12] and surgical resection. [13] Rapid improvement and/or complete resolution after dental treatment have been reported. [14],[15],[16]

Some believe that intralesional triamcinolone (TAC) combined with dapsone yields the best result, whereas others report success using intralesional TAC alone; incidentally TAC has also been successfully combined with clofazimine. [10],[11]

Because of the link between CG and Crohn disease, it has been postulated that tumor necrosis factor-α production is involved in the pathogenesis of both conditions. A single case report describes the successful treatment of CG, which had been refractory to multiple standard therapies, with infliximab at doses that are used for Crohn disease. [17]

Surgical intervention and radiation have been proposed in the management of CG in cases of severe disfigurement and post-surgical relapses are common. [18]


   Conclusion Top


According to new concepts, "diagnosis" is not merely naming the disease but should also include probable etiological factor/s. In this case diagnosis of CG was made by correlation of patient's history, clinical features, investigations and histopathological findings.The present case highlights the importance of thorough investigations in the diagnosis of this lesion as the findings mimic many other granulomatous conditions.

 
   References Top

1.Worsaae N, Christensen KC, Schiødt M, Reibel J. Melkersson-Rosenthal syndrome and cheilitis granulomatosa. A clinicopathological study of thirty-three patients with special reference to their oral lesions. Oral Surg Oral Med Oral Pathol 1982;54:404-13.  Back to cited text no. 1
    
2.van der Waal RI, Schulten EA, van de Scheur MR, Wauters IM, Starink TM, van der Waal I. Cheilitis granulomatosa. J Eur Acad Dermatol Venereol 2001;15:519-23.  Back to cited text no. 2
    
3.Wiesenfeld D, Ferguson MM, Mitchell DN, MacDonald DG, Scully C, Cochran K, et al. Oro-facial granulomatosis: A clinical and pathological analysis. Q J Med 1985;54:101-13.  Back to cited text no. 3
    
4.Muhammed K, Nandakumar G, Thomas S. Granulomatous cheilitis evolving into Melkersson-Rosenthal syndrome with bilateral facial palsy. Indian J Dermatol Venereol Leprol 2004;70:313-4.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
5.Scully C, Langdon J, Evans J. Marathon of eponyms: 13 Melkersson-Rosenthal syndrome. Oral Dis 2010;16:707-8.  Back to cited text no. 5
    
6.Muellegger RR, Weger W, Zoechling N, Kaddu S, Soyer HP, El Shabrawi-Caelen L, et al. Granulomatous cheilitis and Borrelia burgdorferi: Polymerase chain reaction and serologic studies in a retrospective case series of 12 patients. Arch Dermatol 2000;136:1502-6.  Back to cited text no. 6
    
7.Zimmer WM, Rogers RS 3 rd , Reeve CM, Sheridan PJ. Orofacial manifestations of Melkersson-Rosenthal syndrome. A study of 42 patients and review of 220 cases from the literature. Oral Surg Oral Med Oral Pathol 1992;74:610-9.  Back to cited text no. 7
    
8.Olivier V, Lacour JP, Castanet J, Perrin C, Ortonne JP. Cheilitis granulomatosa in a child. Arch Pediatr 2000;7:274-7.  Back to cited text no. 8
    
9.Inui S, Itami S, Katayama I. Granulomatous cheilitis successfully treated with roxithromycin. J Dermatol 2008;35:244-5.  Back to cited text no. 9
    
10.Kato T, Tagami H. Successful treatment of cheilitis granulomatosa with tranilast. J Dermatol 1986;13:402-3.  Back to cited text no. 10
    
11.Bacci C, Valente ML. Successful treatment of cheilitis granulomatosa with intralesional injection of triamcinolone. J Eur Acad Dermatol Venereol 2010;24:363-4.  Back to cited text no. 11
    
12.Allen CM, Camisa C, Hamzeh S, Stephens L. Cheilitis granulomatosa: Report of six cases and review of the literature. J Am Acad Dermatol 1990;23:444-50.  Back to cited text no. 12
    
13.Camacho F, García-Bravo B, Carrizosa A. Treatment of Miescher's cheilitis granulomatosa in Melkersson-Rosenthal syndrome. J Eur Acad Dermatol Venereol 2001;15:546-9.  Back to cited text no. 13
    
14.Takeshita T, Koga T, Yashima Y. Case repot: Cheilitis granulomatosa with periodontitis. J Dermatol 1995;22:804-6.  Back to cited text no. 14
    
15.Kawakami T, Fukai K, Sowa J, Ishii M, Teramae H, Kanazawa K. Case of cheilitis granulomatosa associated with apical periodontitis. J Dermatol 2008;35:115-9.  Back to cited text no. 15
    
16.Lazarov A, Kidron D, Tulchinsky Z, Minkow B. Contact orofacial granulomatosis caused by delayed hypersensitivity to gold and mercury. J Am Acad Dermatol 2003;49:1117-20.  Back to cited text no. 16
    
17.Barry O, Barry J, Langan S, Murphy M, Fitzgibbon J, Lyons JF. Treatment of granulomatous cheilitis with infliximab. Arch Dermatol 2005;141:1080-2.  Back to cited text no. 17
    
18.Kruse-Lösler B, Presser D, Metze D, Joos U. Surgical treatment of persistent macrocheilia in patients with Melkersson-Rosenthal syndrome and cheilitis granulomatosa. Arch Dermatol 2005;141:1085-91.  Back to cited text no. 18
    

What is new? Comprehensive approach in differential diagnosis of granulomatous lesions is highlighted. This is important in prompt treatment planning and better prognosis


    Figures

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



 

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    Abstract
   Introduction
   Case Report
   Discussion
   Conclusion
    References
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