Chelitis Granulomatosa: Clinical Case Report  

Naresh Gangeshetty
Department of Oral Medicine and Radiology, G. Pulla Reddy Dental College and Hospital, Kurnool, India
Author    Correspondence author
International Journal of Clinical Case Reports, 2016, Vol. 6, No. 7   doi: 10.5376/ijccr.2016.06.0007
Received: 22 Oct., 2015    Accepted: 29 Nov., 2015    Published: 30 Dec., 2015
© 2016 BioPublisher Publishing Platform
This is an open access article published under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Preferred citation for this article:

Naresh Gangeshetty, 2016, White Spot Lesions, International Journal of Clinical Case Reports, 6(7) 1-3 (doi: 10.5376/ijccr.2016.06.0007)

Abstract

Chelitis granulomatosa (CG) is a rare disorder characterized by abnormal swelling of the lips which is chronic in nature and it is caused by granulomatous inflammation. This disease may appear independently or may be associated with a Melkersson- Rosenthal syndrome. Here we report a case of chelitis granulomatosa in a 19 year old boy which was successfully treated with intralesional steroid injections.

Keywords
Orofacial granlomatosis; Chelitis granulomatosis; Intralesional steroids

Introduction
Chelitis granulomatosa (CG) is a rare inflammatory disorder first described by Miescher in 1945 (Neeraj et al., 2013). It is characterized by recurrent swelling of the lips and is considered as a monosymptomatic form of Melkersson - Rosenthal syndrome. Melkersson - Rosenthal syndrome is a triad of edema of the lips, facial palsy, and fissured tongue (Martínez et al., 2012). Orofacial granulomatosis is a term used by wiesenfeld in 1985 to describe various diseases of noninfectious, non-necrotizing granulomatous disorders of the lips, face and oral cavity that included Cheilitis granulomatosa (Martínez et al., 2012; Neeraj et al., 2013). Cheilitis Granulomatosa is clinically characterized by the diffuse increase of the lips (one or both), rarely followed by the swelling of the gingiva, the buccal mucosa and the palate (Gojkov-Vukelic et al., 2011). The swelling of the lips is chronic in nature, non-pitting, non-tender and varies in consistency from soft to rubbery (Neeraj et al., 2013). The present paper presents a case of chelitis granulomatosa in a 19 year old male patient which was successfully treated with intralesional steroids.

Case report
A 19 year old male patient presented to the department of oral medicine and radiology of our college with a chief complaint of swelling of the lips and gums since 2 years. History reveals that patient was apparently asymptomatic 2 years back. Initially patient had burning sensation in the lips and gums which were followed by formation of vesicles over them which are ruptured with resultant pus discharge later swelling of the lips and gums started which is initially small and gradually increased in size. The gingival enlargement is seen with the marginal and attached gingiva involving the upper and lower anterior teeth. The swelling then subsided by medication initially but later it is become recurrent in these 2 years. Patient had consulted many doctors with no improvement in his condition. His medical history is normal but there is a family history of diabetes. Patient has no deleterious habits.

On clinical examination, there are no abnormal findings with his systemic condition. As far as teeth are concerned there is missing teeth with left upper central incisor with dental caries with upper right first molar. Soft tissues are normal with swelling of upper and lower lips and gingiva of maxillary and mandibular anterior regions. Both upper and lower lips are enlarged with loss of lip contour and they are everted, erythematous, fissured, and soft to firm in consistency and non-tender without any evidence of bleeding from the lips. The marginal and attached gingiva of maxillary and mandibular anterior teeth are enlarged which results in loss of contour. The enlarged gingiva is soft to firm in consistency, non-tender and overlaps the anterior teeth.

On the basis of history of recurrent swellings and upon examination we have given provisional diagnosis of chelitis granulomatosa with differentials include Melkersson- Rosenthal syndrome, crohn’s disease, sarcoidosis, angioneurotic edema, chelitis glandularis. We subjected patient to various investigations like complete blood picture, bleeding and clotting time, erythrocyte sedimentation rate (ESR), screening for HIV and hepatitits, stool examination. Patient was then subjected for incisional biopsy from lips and gingiva.

Histopathological examination of the tissue section shows inflamed connective tissue and overlying stratified squamous epithelium. The connective tissue shows granuloma formations showing lymphocytes, plasma cells, macrophages and few giant cells of Langerhans type. Numerous plump endothelial cells and budding capillaries are also seen. The overlying epithelium shows irregular proliferation and few areas of ulceration.

Based on clinical examination of lesion, histopathology and excluding other diseases by various investigations, we finally given the diagnosis of Chelitis granulomatosa. Further patient was referred to gastroenterologist to exclude other Granulomatous disease like crohn’s disease. Gastroenterologist advised for endoscopy and colonoscopy and has gone for the duodenal, Ileal and rectal biopsy. The endoscopy, colonoscopy and biopsy from the duodenum, ileaum and rectum have not showed any specific lesion. Chelitis granulomatosa has been treated with intralesional steroid injection of 2 mL of dexamethasone admixed with 0.5 mL of lignocaine. The injections were given at an interval of 1 week for about 1 month. Patient underwent Oral prophylaxis before the start of steroid therapy to decrease the inflammation of the gingiva. The lesions were healed after 1 month of treatment and were followed for about 6 months and patient has not experienced any lesions within this period.

Discussion
Chelitis Granulomatosis (CG) refers to persistent, painless swelling confined to one or both lips (Neeraj et al., 2013; Baqai et al., 2013). The term Orofacial granulomatosis (OFG) was first introduced by Wiesenfeld in 1985. It refers to a rare condition of unknown aetiology which is used to describe swelling in the lips, face or areas within the mouth, in the absence of any recognised systemic conditions (Baqai et al., 2013).

The exact etiology of Chelitis granulomatosis (CG) is unknown. Several reports have shown that CG has been associated with infection, genetic, allergic reactions, odontogenic foci, autoimmune mechanism or as an association with Crohn’s disease and sarcoidosis or as an oral manifestation of systemic disease (Martínez et al., 2012; Neeraj et al., 2013; Gupta and Singh, 2014). Granulomatous cheilitis is a rare disease. It can appear at any age, but is most frequently seen in the second or third decade of lifewith no racial or sexual predilection (Martínez et al., 2012; Neeraj et al., 2013; Vibhute et al., 2013; Gupta and Singh, 2014).

Cheilitis Granulomatosa is clinically characterized by the diffuse increase of either one or both the lips, followed by the swelling of the gingiva and rarely of buccal mucosa and the palate (López-Urbano et al., 2009). The disease can appear either independently or it may be linked to Facial paralysis and lingua plicata which are then characteristic of the Melkersson-Rosenthal Syndrome (Gojkov-Vukelic et al., 2011). The facial paralysis may be unilateral or bilateral; partial or complete and the fissured tongue or lingua plicata may be associated with burning sensation (Agarwal et al., 2011). The presence of Chelitis Granulomatosa without lingua plicata or facial paralysis is also called as Miescher syndrome or Miescher's Cheilitis (Neeraj et al., 2013). Most commonly Chelitis Granulomatosa presents as an acute, asymptomatic swelling of the lips which lasts for hours to days and the episodes of swelling are self-limiting initially which resolve completely in later stages. The episodes of swelling may be associated with fever and mild constitutional symptoms such as headache and visual disturbances. After several episodes the lips become firm and indurated. Patients may report pain or a burning sensation if there is erythema, fissures and erosions on lips and sometimes we may see scaling of the lips (Martínez et al., 2012; Baqai et al., 2013).

Diagnosis of cheilitis granulomatosa relies on the typical history, clinical features and presence of non-caseating granuloma on histopathology (Martínez et al., 2012; Bhushan et al., 2012). Various other diseases should be considered in differential diagnosis which include angioedema (idiopathic or hereditary), sarcoidosis, Crohn′s disease, some specific infections (tuberculosis, leprosy, and deep fungal infections), Amyloidosis, certain soft-tissue tumors, minor salivary gland tumor, foreign body giant cell granuloma (Chandan et al., 2012; Kavya et al., 2013; Gupta and Singh, 2014). Histopathological examination of lesions may reveal non necrotizing granulomas, edema, lymphangiectasia, and perivascular lymphocytic infiltration. But it has been suggested that these histological features may not evident in the initial stages of the disease (Martínez et al., 2012; Bhushan et al., 2012).

Treatment of Chelitis granulomatosa is a challenge to the patient and many drugs have been tried for the treatment of this disease. Various drugs used in treatment include tetracycline, clofazimine, systemic, topical, intralesional steroids, systemic antibiotics, hydroxychloroquine, sulfasalazine, antihistamines, metronidazole, macrolides, thalidomide and infliximab (Gojkov-Vukelic et al., 2011; Martínez et al., 2012; Neeraj et al., 2013). Intralesional injections of Triamcinolone or combination therapy of intralesional triamcinolone with clofazimine or dapsone are the most commonly used treatment options (Martínez et al., 2012; Bhushan et al., 2012; Vibhute et al., 2013). Intralesional triamcinolone acetonide with lignocaine 2 percent is the first line of treatment for cheilitis granulomatosa. Intralesional triamcinolone acetonide (10-20 mg/mL) in the volume of 1.0~1.5 mL into each side of the lip leads to prompt resolution of inflammatory granulomas (Bacci and Valente, 2010; Bhushan et al., 2012). Rapid improvement or complete resolution is seen in patients who underwent dental treatment along with intralesional injection of steroids (Martínez et al., 2012). Surgery is indicated for patients who do not respond to steroid therapy or who present a reasonable face deformation (Agarwal et al., 2011; Bhushan et al., 2012).

In Conclusion, a rare case of Cheilitis Granulomatosa in a 19 year old patient is presented. The case presented here has swelling of lower lips and gingivae which are recurrent and histopathology shows granulomas and it is successfully treated with intralesional dexamethasone.

Author’s contribution
Naresh Gangeshetty has completely taken care of the case. He has done the examination, taken biopsy and treated the case.

Acknowledgements
I would like to thank my guide and teacher Dr. Venkateswarulu for guiding me in treating the patient.

References
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