Indian Dermatology Online Journal

THROUGH THE LENS
Year
: 2016  |  Volume : 7  |  Issue : 6  |  Page : 561--562

Cheilitis granulomatosa


Sukumaran Pradeep Nair 
 Department of Dermatology and Venereology, Government Medical College, Trivandrum, Kerala, India

Correspondence Address:
Sukumaran Pradeep Nair
Department of Dermatology and Venereology, Government Medical College, Trivandrum - 695 011, Kerala
India




How to cite this article:
Nair SP. Cheilitis granulomatosa.Indian Dermatol Online J 2016;7:561-562


How to cite this URL:
Nair SP. Cheilitis granulomatosa. Indian Dermatol Online J [serial online] 2016 [cited 2019 Nov 23 ];7:561-562
Available from: http://www.idoj.in/text.asp?2016/7/6/561/193914


Full Text

Cheilitis granulomatosa (CG), also known as Miescher's cheilitis, is a recurrent or chronic firm swelling of the lips of unknown etiology mimicking angioedema, but persistent. The triad of labial swelling, facial palsy, and scrotal tongue (lingua plicata) is termed Melkersson–Rosenthal syndrome (M–R).[1] The presentation with only labial edema is considered to be the monosymptomatic form of the above syndrome.

A 46-year-old man presented with persistent lower lip edema of 14 years duration with negative history of any precipitating factor. On examination there was diffuse, firm, non-tender, erythematous, edematous swelling of the lower lip with areas of hyperpigmentation, depigmentation, and scaling along the lower vermillion border [Figure 1]. There was no facial palsy or scrotal tongue. The rest of the face was normal. There was no cervical lymphadenopathy and systemic examination was unremarkable. The patient's blood and urine routine test results were within normal limits. Serum calcium and ACE (angiotensin converting enzyme) levels were within normal limits. Chest radiography and high resolution computed tomography scan of the lungs was normal and did not show any hilar lymphadenopathy. Endoscopy did not show any evidence of inflammatory bowel disease. Biopsy of the lower lip showed a few scattered epithelioid cell noncaseating granulomas, lymphocytes, and no giant cells [Figure 2]. Staining for AFB (acid fast bacilli) and fungus were negative. Culture of the tissue for AFB and fungus were also negative. Thus a final diagnosis of CG was made. The absence of facial palsy and scrotal tongue qualifies this case as the monosymptomatic form of M–R syndrome. The patient is being planned for cheiloplasty by the plastic surgeons.{Figure 1}{Figure 2}

CG is a chronic benign condition presenting with persistent firm lip edema. The reports of it being a localized form of sarcoidosis and Crohn's disease is only anecdotal.[2] In our case, the investigations and biopsy did not show any evidence of the aforementioned conditions. There have been reports of CG being a granulomatous reaction to cobalt and food addictives such as cinnamic aldehyde.[3] In addition to the lips, the cheeks, eyelids, forehead, scalp, and genitals can also be affected. Associated regional lymphadenopathy may be present. The early biopsy may show only perivascular lymphocytic infiltrate and later a few noncaseating granulomas may be seen. There is no definite treatment. Cheiloplasty, intralesional triamcinolone, clofazimine, metronidazole, thalidomide, suphasalazine, and infliximab are the various treatments reported.[4],[5] Spontaneous regression has also been mentioned.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Khandpur S, Malhotra AK, Khanna N. Melkersson-Rosenthal syndrome with diffuse facial swelling and multiple cranial nerve palsies. J Dermatol 2006;33:411-4.
2Khouri JM, Bohane TD, Day AS. Is orofacial granulomatosis in children a feature of Crohn's disease? Acta Paediatr 2005;94:501-4.
3McKenna KE, Walsh MY, Burrows D. The Melkersson-Rosenthal syndrome and food additive hypersensitivity. Br J Dermatol 1994;131:921-2.
4Kruse-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.
5Coskun 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.