Cheilitis granulomatosa.

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

Firm diffuse erythematous edematous swelling of lower lips

Figure 2

Biopsy showing granulomas with epithelioid cells (arrows) and lymphocytes, H and E × 400

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.[45] Spontaneous regression has also been mentioned.

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