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CASE REPORT
Year : 2017  |  Volume : 18  |  Issue : 3  |  Page : 217-219

Idiopathic granulomatous cheilitis of Miescher in a young patient: A rare entity and its successful treatment


Department of Dermatology, Bangalore Medical College and Research Institute, Bengaluru, Karnataka, India

Date of Web Publication7-Jun-2017

Correspondence Address:
Bhumika Shivaram
Department of Dermatology, Bangalore Medical College and Research Institute, Fort, KR Road, Bengaluru - 560 002, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2319-7250.188453

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  Abstract 

Granulomatous cheilitis (GC) of Miescher is a chronic inflammatory disorder of unknown etiology affecting young adults, characterized by asymptomatic, unrelenting swelling of lips. An array of treatment modalities have been tried, only for the disease to recur again. We report a case of GC with significant remission after treatment with a combination of steroids, metronidazole, and doxycycline. There were no signs of relapse at 6 months of follow-up.

Keywords: Doxycycline, granulomatous cheilitis, intralesional-steroids, metronidazole


How to cite this article:
Shanmukhappa AG, Shivaram B, Budamakuntala L, Samynathan A. Idiopathic granulomatous cheilitis of Miescher in a young patient: A rare entity and its successful treatment. Indian J Paediatr Dermatol 2017;18:217-9

How to cite this URL:
Shanmukhappa AG, Shivaram B, Budamakuntala L, Samynathan A. Idiopathic granulomatous cheilitis of Miescher in a young patient: A rare entity and its successful treatment. Indian J Paediatr Dermatol [serial online] 2017 [cited 2017 Sep 26];18:217-9. Available from: http://www.ijpd.in/text.asp?2017/18/3/217/188453


  Introduction Top


Granulomatous cheilitis (GC) is a unique inflammatory disorder first described by Miescher in 1945, characterized by persistent swelling of either or both lips with noncaseating granulomatous infiltration.[1] The term, idiopathic GC is used to describe cases without a known etiology, thus rendering this condition difficult to treat.[2] The uniqueness of this case report being the unremarkable sustained remission seen with a combination treatment of intralesional steroids, metronidazole, and doxycycline.


  Case Report Top


A 9-year-old boy reported in our outpatient Department of Dermatology at Bangalore Medical College, Bengaluru with an 18-month history of persistent, unremitting swelling of the upper lip. The swelling was first noticed in the center of upper lip which gradually progressed to involve the whole of exposed part of upper labia. Relevant history of fever, gastrointestinal symptoms such as abdominal cramps, diarrhea, blood in stools and fatigue or weight loss was absent. Allergic component to any food or drug was not present. Systemic examination was normal. Pain or bleeding from the swelling was not noticed. History of receiving intralesional steroids with partial remissions and reappearances was present.

On examination, an erythematous, diffuse, firm, asymptomatic swelling of the upper lip was noticed [Figure 1]. There was no local rise of temperature or tenderness. Signs of bleeding, exudation or scabs were not found on the surface of the swelling. Intraoral examination revealed mild enlargement of gingiva in the maxillary region with good dentition. Fissuring of the tongue, cobblestone appearance of oral mucosa or oral ulcers was not present. There was no evidence of facial nerve palsy. Regional lymph nodes were not enlarged.
Figure 1: Pretreatment photograph showing diffuse swelling of upper lip

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Routine investigations including complete hemogram, peripheral blood smear, erythrocyte sedimentation rate, liver function test, blood urea nitrogen, and serum creatinine were performed, which were within the normal range. Serum levels of angiotensin-converting enzyme and serum calcium levels were also within normal limits, thus ruling out sarcoidosis. With a normal chest X-ray and a negative intradermal Mantoux test, tuberculosis was ruled out. Since stool for occult blood was negative, and the patient lacked any gastrointestinal symptoms, further investigations as endoscopy for Crohn's disease was not done. Patch testing was not done because an allergy to any food, drug, or dental materials was not found. An incisional biopsy of the enlarged upper lip revealed a histopathological picture of noncaseating epithelioid granulomas with the rim of lymphocytes in the dermis, confirming our diagnosis of GC [Figure 2] and [Figure 3].
Figure 2: Histopathological picture of H and E staining showing perivascular infiltration of lymphocytes, Langhans giant cells, epithelioid cells and noncaseating granulomas (low power view)

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Figure 3: Histopathological picture of H and E staining showing perivascular noncaseating granulomas (high power view)

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We started a treatment protocol of intralesional triamcinolone acetonide 10 mg/ml (0.25 ml) at three equidistant points between mucosa and upper lip vermillion, given once a week for 1 month.[2] Alongside, oral metronidazole 200 mg (20–30 mg/kg/day) thrice a day and oral doxycycline 50 mg (2–3 mg/kg/day) once in the night was prescribed for 1 month. Oral doxycycline is considered safe in children above 9 years of age without adverse effects of staining teeth or bone abnormalities. The patient tolerated the regimen well. There was a significant reduction in the size of the swelling in 15 days from the start of treatment. The swelling completely disappeared leaving behind pigmentation of upper lip at the end of 1 month [Figure 4]. As a maintenance, only oral doxycycline 50 mg once a day on alternate days was given for another month period. There was a recurrence of swelling of upper lip after 2 months which subsided after two intralesional triamcinolone acetonide 10 mg/ml injections each given at an interval of 1 week. The patient was followed up for 6 months, with no evidence of recurrence.
Figure 4: Posttreatment photograph showing significant reduction in swelling of upper lip after 6 months

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  Discussion Top


GC is a granulomatous condition, manifesting as chronic, recurrent swelling of one or both the lips.[1] When GC is accompanied by facial muscle palsy and fissured tongue, it is known as Melkerson–Rosenthal syndrome.[4] This classical triad is very uncommon, representing only 25–40% of cases.[5] The monosymptomatic form of GC is referred to as Miescher's cheilitis.

The etiology of GC is precisely not known.[3] Various theories have been hypothesized, including genetics, infections, allergy to food, drugs, and dental materials and autoimmune disorders.[6] Further suggestions as monoclonal lymphocyte expression, occurring due to chronic antigenic stimulation resulting in cytokine production which forms granulomas have also been given.[3] Systemic diseases such as Crohn's disease and Sarcoidosis may manifest with GC.[2] Other differentials to be excluded are tuberculosis, leprosy, foreign body reactions and systemic fungal infections, amyloidosis, soft-tissue tumors, Wegener's granulomatosis, angioedema and minor salivary gland tumors.[3] GC may present as an early manifestation as Crohn's disease, it may follow, coincide, or precede with the onset of Crohn's disease.[7] Investigations such as endoscopy and colorectal biopsy are justified only when gastrointestinal symptoms or signs are evident.[8] Thus, in our case, we have ruled out other causes of GC by relevant clinical examination and investigations.

Histopathological examination shows perivascular infiltration of lymphocytes, plasma cells and noncaseating granulomas which are clustered around scattered blood vessels with Langhans giant cells and epithelioid cells.[9] The histopathological features also favor our present case diagnosis.

The treatment of idiopathic GC is a tough challenge as recurrences are common with most of the available treatment modalities. Spontaneous resolution is rare but has been reported. Many different drugs have been used, including intralesional steroids, oral prednisone, antimalarials, minocycline, tetracycline, metronidazole, adalimumab, infliximab, and surgical measures as cheiloplasty; however, comparative trials are lacking.[10]

Various combination regimens showing successful results have been reported, such as, Intralesional steroids and metronidazole by Coskun et al. and oral prednisolone and minocycline by Stein and Mancini.[3] Significant improvement was seen with a combination treatment of intralesional triamcinolone, minocycline, and metronidazole, as reported by Dar et al.[3]

Considering intralesional corticosteroid as the first line of management and the combination of antibiotics resulting in a potent anti-inflammatory mechanism of action, a similar regimen followed by Dar et al. was given in our patient. A dramatic response was seen, even though recurrences are common, it can be kept at bay by intralesional steroids.

To conclude, the diagnosis of GC is supported by histopathological evidence of chronic granulomatous inflammation. Intralesional-corticosteroids along with a combination of antibiotics are beneficial. However, a regular follow-up and review should be advised.

Financial Support and Sponsorship

Nil.

Conflicts of Interest

There are no conflicts of interest.

 
  References Top

1.
Vibhute NA, Vibhute AH, Daule NR. Cheilitis granulomatosa: A case report with review of literature. Indian J Dermatol 2013;58:242.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Rangdhol RV, Madhulika N, Dany A, Jeelani S, Asokan GS. Idiopathic orofacial granulomatosis – A diagnostic and treatment challenge. J Clin Diagn Res 2014;8:ZD07-10.  Back to cited text no. 2
[PUBMED]    
3.
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. 3
    
4.
Critchlow WA, Chang D. Cheilitis granulomatosa: A review. Head Neck Pathol 2014;8:209-13.  Back to cited text no. 4
    
5.
Brar BK, Mahajan BB, Kamra N. Cheilitis granulomatosa (Miescher granulomatous macrocheilitis) with Down syndrome: A rare alliance. Indian J Paediatr Dermatol 2016;17:50-2.  Back to cited text no. 5
  [Full text]  
6.
Rachisan AL, Hrusca A, Gheban D, Cainap S, Pop TL, Baican A, et al. Granulomatous cheilitis of Miescher: The diagnostic proof for a Melkersson-Rosenthal syndrome. Rom J Morphol Embryol 2012;53 3 Suppl: 851-3.  Back to cited text no. 6
    
7.
Triantafillidis JK, Valvi FZ, Merikas E, Peros G, Galitis ON, Gikas A. Granulomatous cheilitis associated with exacerbations of Crohn's disease: A case report. J Med Case Rep 2008;2:60.  Back to cited text no. 7
    
8.
Kaarthikeyan G, Arvind M, Jayakumar N, Khakar M. Idiopathic orofacial granulomatosis in a young patient: A rare entity. J Oral Maxillofac Pathol 2012;16:432-4.  Back to cited text no. 8
[PUBMED]  [Full text]  
9.
Denny EC, Ashok L, Shivaprasad S, Anitha B, Mujib A. Cheilitis granulomatosa. JIAOMAR 2006;18:167-9.  Back to cited text no. 9
    
10.
López-Urbano MJ, Villar MA, Ruiz Villaverde R, Avila IR. Granulomatous cheilitis: A condition that merits inclusion in the differential diagnosis of angioedema. J Investig Allergol Clin Immunol 2009;19:68-70.  Back to cited text no. 10
    


    Figures

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



 

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