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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 37  |  Issue : 2  |  Page : 85-88

Granulomatous cheilitis involving the lower lip


Department of Dermatology & Venereology, Pramukshwami Medical College, Karamsad, Gujarat, India

Date of Submission27-Feb-2017
Date of Acceptance17-Apr-2017
Date of Web Publication4-Aug-2017

Correspondence Address:
Pragya A Nair
Department of Dermatology & Venereology, Pramukshwami Medical College, Karamsad, Gujarat 388325
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ejdv.ejdv_8_17

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  Abstract 


Granulomatous lesions of the oral and oropharyngeal submucosal tissues refer to painless, idiopathic swelling frequently affecting the buccal and labial areas. They present a diagnostic dilemma because of the wide variety of possible etiologic factors. Granulomatous cheilitis should be considered in the differential diagnosis of persistent lip swelling. It can occur in isolation as a monosymtomatic form or as a part of Melkersson–Rosenthal syndrome, which is a triad of recurrent orofacial swelling, relapsing facial paralysis, and fissuring of the tongue (lingua plicata). Spontaneous remission is rare, and recurrences are common. Corticosteroids used for treatment provide temporary improvement. A combination of intralesional triamcinolone and clofazimine or dapsone is one of the most commonly used treatment options. We present the case of a 21-year-old young man with granulomatous cheilitis who responded to intralesional steroids and dapsone.

Keywords: cheilitis, dapsone, granuloma, lips


How to cite this article:
Nair PA, Patel TM. Granulomatous cheilitis involving the lower lip. Egypt J Dermatol Venerol 2017;37:85-8

How to cite this URL:
Nair PA, Patel TM. Granulomatous cheilitis involving the lower lip. Egypt J Dermatol Venerol [serial online] 2017 [cited 2017 Nov 24];37:85-8. Available from: http://www.ejdv.eg.net/text.asp?2017/37/2/85/212106




  Introduction Top


Orofacial granulomatosis (OFG) is a rare condition first described by Wiesenfeld in 1985. It is described by swelling of the lips, face, or areas within the mouth, caused by noncaseating granulomatous inflammation in the absence of systemic disease [1].

Granulomatous cheilitis (GC) is a rare, persistent, painless, idiopathic chronic swelling of the lips, which is considered as a manifestation of OFG. GC has no predisposition to race, sex, or age, and its incidence has been estimated at 0.08% in the general population [2]. It can occur in isolation as a monosymtomatic form or as a part of Melkersson–Rosenthal syndrome (MRS), which is a triad of recurrent orofacial swelling, relapsing facial paralysis, and fissuring of the tongue (lingua plicata) .GC should be considered in the differential diagnosis of persistent lip swelling. Treatment options include dietary modifications, antibiotics, systemic or intralesional corticosteroids, and surgery.


  Case report Top


A 21-year-old man presented to our Dermatology Department with left-sided, lower-lip swelling since 7 months, which was gradually progressive. There were no complaints of itching or pain. There were no lesions elsewhere over the body. On palpation, lower-lip swelling ([Figure 1]) was present, which was nontender and soft with normal temperature .There was no gingival swelling. There was no history of any drug intake/trauma/insect bite/teeth pain or pus discharge. He had no history of angioedema, anaphylaxis, seasonal allergic rhinitis, or any malignancy . There was no family history of angioedema. All routine investigations were normal. A punch biopsy was obtained from the lower lip, which showed mild acanthosis of the epidermis and scattered, noncaseating, poorly formed granuloma comprised of epithelioid cells and multinucleated giant cells with lymphoplasmacytic inflammatory infiltrate in the dermis. Perivascular lymphocytic infiltration was also present, and changes were consistent with diagnosis of GC ([Figure 2]a and [Figure 2]b). The patient was administered intralesional triamcinolone acetonide 10 mg with an insulin syringe to the lower lip, and oral dapsone 100 mg once a day was administered after determining glucose-6-phosphate dehydrogenase levels. The swelling reduced after 15 days. Intralesional injections were repeated after 3 weeks for three sessions, and dapsone was continued. The swelling reduced significantly after 3 months ([Figure 3]).
Figure 1 Swelling on the lower lip.

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Figure 2 (a) Mild acanthosis of the epidermis and scattered, noncaseating, poorly formed granuloma comprised of epithelioid cells and multinucleated giant cells with lymphoplasmacytic inflammatory infiltrate in the dermis (hematoxylin and eosin stain, ×10). (b) Granuloma comprised of epithelioid cells and multinucleated giant cells (hematoxylin and eosinstain, ×40).

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Figure 3 Reduced swelling after 3 months.

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  Discussion Top


OFG is an umbrella term that comprises entities with diverse etiologies, pathogenesis, and clinical features. GC was first described by Miescher in 1945 [3].

It refers to persistent, chronic, relapsing, remitting course of painless swelling confined to one or both lips. The cosmetic deformity leads to significant psychological impact on a young patient. GC usually affects young adults, mostly in the second decade of life with a female predilection [4].

The etiology of GC is poorly understood but has been associated with genetic, allergic, and infectious causes [5],[6]. A variety of foods including wheat and dairy products, chocolate, eggs, peanuts, cocoa, cinnamaldehyde, monosodium glutamate, cormosine, food additives, toothpaste, and dental material such as amalgam and mercury has been suggested in several case reports [7]. It could present as an oral manifestation of Crohn’s disease [8]. Hornstein suggested that the disorder is polyetiologic and may be caused by an alteration in autonomic nervous system function localized to facial skin, resulting in increased vascular permeability and edema [4]. A current hypothesis is that it is due to random influx of inflammatory cells and not a specific, single antigen [6].

Monoclonal lymphocytic expression, secondary to chronic antigenic stimulation, cytokine production leading to granuloma formation, and a cell-mediated hypersensitivity reaction have also been suggested [6]. When accompanied by facial palsy and plicated tongue, it is referred to as the MRS.

Clinically, the first episode typically subsides in hours or days, but both frequency and duration of the attacks increase until they become persistent. The upper lip, lower lip, or both lips can be involved. The disease can also affect other oral and facial regions including the face, oral mucosa, gums, tongue, pharynx, and larynx. Patients may complain of pain or a burning sensation, especially if the oral involvement is associated with erythema, fissures, erosions, or scaling of the lips, which may retain some degree of firm and indurated edema. The oral mucosa is thickened and edematous with the buccal and labial (70%) mucosa assuming a corrugated or lobulated appearance. Occasionally, palatal involvement has the appearance of papillary hyperplasia. A characteristic gingival hyperplasia when present has a patchy distribution and a predilection for the anterior region. If other areas of face are involved, there may be enlarged regional lymph nodes. The tongue may develop fissures, edema, paresthesia, erosions, or taste alterations. Cobblestone appearance of the buccal mucosa may be observed. The palate may have papules or hyperplastic tissue [9].

The differential diagnosis of persistent upper lip swelling includes other granulomatous diseases such as a foreign body reaction, mycobacterial infection, sarcoidosis, Crohn’s disease, Wegener’s granulomatosis, and histoplasmosis; amyloidosis; rosacea; medications such as angiotensin-converting enzyme inhibitors and calcium channel blockers; atopic reaction to a wide variety of allergens; and hereditary diseases such as C1 esterase deficiency [10].

Diagnosis of GC is made by ruling out other conditions. History of atopy, drug history (angiotensin-converting enzyme inhibitors and calcium channel blockers should alert the physician to the possibility of angioedema), and family history of hereditary angioedema are important to rule out other causes for the swelling. For swelling lasting more than 2 weeks, causes for chronic cough (e.g. sarcoidosis and histoplasmosis) have to be ruled out. Patients without dental infections who present with clinical features suggestive of CG should be questioned regarding the presence of systemic signs and symptoms of Crohn’s disease, sarcoidosis, or a history of angioedema [4].

The initial evaluation must include an assessment of stridor or laryngeal swelling, as these can be a medical emergency. The primary feature that should help diagnose angioedema from other causes is a complete resolution of symptoms between attacks.

A family history of similar orofacial swelling should prompt an investigation into the autosomal dominant C1 esterase inhibitor deficiency, also known as hereditary angioedema.

History taking should include gastrointestinal symptoms, which may alert the dermatologist to suspect Crohn’s disease and to refer accordingly. History suggestive of chronic chough may indicate sarcoidosis, tuberculosis, or histoplasmosis [11].

Physical examination should include a cranial nerve examination to determine facial nerve involvement, a full oropharyngeal examination to check poor dentition or lesions concerning malignancy, examination of the tongue for plication to diagnose MRS, inspection for aphthous ulcers that may suggest early Crohn’s disease, and inspection for erythematous lesions suggestive of rosacea.

In our case, there was involvement of the lower lip, although the upper lip is the most common site of occurrence according to the literature.

GC usually affects the upper lip and, less frequently, the lower lip. However, in a study by Martinez et al. [10], two (33.33%) patients had swelling on other parts of the face − one had swelling over the gums and upper lip, and the other had swelling in perioral lesions, cheeks, and the submandibular region in addition to the upper lip.

The clinical differential diagnosis for a swollen lip can be extensive, but thorough history taking and careful clinical examination will usually eliminate many diagnostic possibilities. Diagnosis of GC is based on clinical findings confirmed by histology.

Histopathology revealed dilation of lymphatic vessels, perivascular lymphocytic infiltration, and to a varying degree non-necrotizing granulomas in the dermis. Typically, the granulomas appear to cluster around scattered vessels and are not well formed or discrete. Fibrosis may be present in long-term lesions [5].

Spontaneous remission is rare, and recurrences are common. Corticosteroids (local or oral) have been used with temporary improvement. Combination therapy with intralesional triamcinolone and clofazimine or dapsone is one of the most commonly used treatment options [12].

Our patient responded to dapsone and intralesional steroids after 3 months of therapy. Antibiotics such as sulfa drugs, tetracycline, macrolides, minocycline, roxithromycin, hydroxychloroquine, sulfasalazine, antihistamines, thalidomide, isoniazid, and metronidazole and immune modulators such as infliximab and methotrexate have been reported to provide good results [13]. Elimination diets have been recommended, such as cinnamon-free and benzoate-free diets. Surgical treatment is an option for patients who are severely affected or have impaired function. Cheiloplasty is reserved for treatment-resistant cases with major deformity

Acknowledgements

The authors acknowledge the contributions of Dr Kirti Rathod and Dr Monica Gupta from the Pathology Department for histopathology diagnosis and photographs.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Grave B, McCullough M, Wiesenfeld D. Orofacial granulomatosis − a 20-year review. Oral Dis 2009; 15:46–51.  Back to cited text no. 1
    
2.
El-Hakim M, Chauvin P. Orofacial granulomatosis presenting as persistent lip swelling: review of 6 new cases. J Oral Maxillofac Surg 2004; 62:11147.  Back to cited text no. 2
    
3.
Miescher G. Uber essentielle granulomatose. Makrocheilie (Cheilitis granulomatosa). Dermatologica 1945; 91:57–85.  Back to cited text no. 3
    
4.
Ceena DE, Ashok L, Shivprasad S, Anitha B, Ahmed Mujib BR. Cheilitis granulomatosa. JIAOMR 2006 18:167–169.  Back to cited text no. 4
    
5.
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; 136:1502–1506.  Back to cited text no. 5
    
6.
Tilakaratne WM, Freysdottir J, Fortune F. Orofacial granulomatosis: review on aetiology and pathogenesis. J Oral Pathol Med 2008; 37:191–195.  Back to cited text no. 6
    
7.
Taibjee S, Prais L, Foulds I. Orofacial granulomatosis worsened by chocolate: results of patch testing to ingredients of Cadbury’s chocolate. Br J Dermatol 2004; 150:595.  Back to cited text no. 7
    
8.
Van de Scheur MR, van der Waal RI, Völker-Dieben HJ, Klinkenberg-Knol EC, Starink TM, van der Waal I. Orofacial granulomatosis in a patient with Crohn’s disease. J Am Acad Dermatol 2003; 49:952–954.  Back to cited text no. 8
    
9.
Goel S, Khorate M, Nahar P, Ahmed J. Cheilitis granulomatosa − an uncommon clinicopathological entity: a case report. J Cancer Sci Ther 2010; 2:086–088.  Back to cited text no. 9
    
10.
Martinez Martinez ML, Azana Defez JM, Perez-Garcia LJ, Lopez-Villaescusa MT, Rodriguez Vazquez M, Faura Berruga C. Granulomatous cheilitis: a report of 6 cases and review of the literature. Actas Dermosifiliogr 2012; 103:718–724.  Back to cited text no. 10
    
11.
Critchlow WA, Chang D. Cheilitis granulomatosa: a review. Head and Neck Pathol 2014; 8:209–213.  Back to cited text no. 11
    
12.
Bacci C, Valente ML. Successful treatment of cheilitis granulomatosa with intralesional injection of triamcinolone. J Eur Acad Dermatol Venereol 2010; 24:363–364.  Back to cited text no. 12
    
13.
Banks T, Gada S. A comprehensive review of current treatments for granulomatous cheilitis. Br J Dermatol 2012; 166:934–937.  Back to cited text no. 13
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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