Teaching Case

A 19-year-old male patient presented with a year-old bilateral lip swelling, upper lip more swollen than lower lip (Fig 1). There is no history of any systemic disease, trauma, allergy, dental problem. On oral examination painless, non-tender, diffuse swelling of both lips observed.

Patient referred to radiology department for imaging of face and lips. The Magnetic Resonance Imaging of the face demonstrated low-signal intensity on T1W sequence (Fig 2) and intermediate to high-signal intensity changes on T2W and STIR sequences (Fig 3 & 4). Gradient echo images indicated no bleed or vascular malformation (Fig 5). Intravenous gadolinium post-contrast study revealed homogeneous significant enhancement of thickened soft tissue of lips (Fig 6).

Accordingly, an incision biopsy of the upper lip was performed aseptically under local anaesthesia. Histopathological examination revealed ill-defined non-caseating granulomas composed of aggregates of epitheloid histiocytes with lymphocytes (Fig 7). The stroma showed dense sclerosis with hyalinisation along with focal lymphocytic infiltration. Furthermore, no evidence of necrosis or dysplasia or malignancy was seen and the section was found to be acid fast bacteria (AFB) negative. Based on clinical, imaging and histopathological evidence it is proved to be Cheilitis granulomatosa.

Cheilitis granulomatosa (CG) is an uncommon chronic granulomatous inflammatory disease characterised by painless swelling of one or both of the lips. Dr. Miesher, a German dermatologist was the first one to describe such lesions as Cheilitis granulomatosa in 1945 [1, 2]. CG has a female predilection, affecting young adults [3]. CG is believed to be a variant of Melkersson Rosenthal Syndrome (MRS) which is a triad of recurrent orofacial soft tissue oedema, tongue plication and facial paralysis. CG is also considered as a manifestation of orofacial granulomatosis (OFG), a notion given by Wiesenfield in the year 1985 [4].

The exact cause of Cheilitis granulomatosa is unknown. The possible aetiologies discussed are hypersensitive reaction, chronic dental infections, food allergy, an association of Crohn's disease, sarcoidosis, systemic disease or hereditary angioedema [3, 5, 6]. According to Hornstein et al. CG could be due to alteration in autonomic nervous system of face leading to increased vascular permeability and oedema [7].

Within the lamina propria non-caseating granulomas with multinucleated giant cells, perivascular lymphocytic aggregates and lymphatic vessels dilatation are the usual histopathological findings associated with Cheilitis granulomatosa [5]. Long standing lesions can cause fibrosis [3].

As the differential diagnosis of lip swelling is quite lengthy, it is very important to identify the causative factor by taking proper history and thorough evaluation. Chest radiograph is done to rule out tuberculosis and sarcoidosis, look for presence of any gastrointestinal symptoms of Crohn's disease, family history of angioedema (C1 esterase inhibitor deficiency) and allergy testing for sensitive food items [5, 6, 8].

Corticosteroids have found to be effective in treating Cheilitis granulomatosa and preventing relapses [5, 9]. Radiation therapy and surgery indicated in cases which show no response to steroids and in gross disfiguring lip swelling [6, 10].
Chymoral Forte has been also proved an effective means of treatment of Cheilitis granulomatosa, thus preventing the patients from unnecessary adverse effects of steroids [11].

In our case, oral as well as intralesional steroids have been given to treat CG, and the patient responded well with no evidence of recurrence. This case report is an attempt aimed to contribute towards available radiological evidence of CG. At present due to non-specificity there are less documentary discussions available on radiological findings of CG. To summarise, as the exact aetiology of CG is not known, a detailed history, investigations along with histopathology will help to make diagnosis and early treatment of Cheilitis granulomatosa, as the features mimic other granulomatous diseases.