A 30-year-old man of African descent came to our attention because of an extremely severe swelling involving both lips (Fig. 1). Such condition began in 2006 with the involvement first of the upper, followed by the lower lip. The disease progressively worsen up to the present. Both past medical and family history were unremarkable. Tuberculosis, sarcoidosis and Crohn's disease were excluded by Chest High-resolution computed tomography (HRCT), colonoscopy and intestinal biopsy. Neurologic examination did not showed peripheral facial nerve palsy. The patient had already experienced several medical treatments including intra-lesional steroid injections (triamcinolone) and systemic steroids (prednisolone) with progression of the lip edema and off-label anti-TNF-alfa therapy (infliximab 5 mg/kg every 8 weeks) which was able to stabilize the disease. Local physical examination reported severe swelling of both lips. The superficial mucosa looked translucent, dry, with paramedian brownish dotting and fissured chaps. Consistency of the lips was firmly elastic, thus to limit facial mimic. Intra oral examination showed no particular tissue abnormalities, neither fissured tongue or lingua plicata. The patient referred lowered local sensation, functional limitation, but, above all, dramatic social embarrassment with a complete lack of confidence.
An incisional biopsy was taken from the upper lip. Histopathological examination showed numerous dilated lymphatic vessels and a mild inflammatory infiltrate, composed of lymphocytes and rare non caseating epithelioid granulomas in the subepithelial connective tissue (Fig. 2), consistent with a diagnosis of Miescher's cheilitis. In vitro studies from a biopsy fragment revealed a mixed CD4+ and CD8+ T lymphocyte population able to produce high levels of Interferon (IFN-)-gamma and Tumor necrosis factor (TNF-)-alfa, consistent with the granulomatous inflammation (data not shown).
After a clinically stable preoperative period of two years with no further increase of lip dimensions, achieved with anti-TNF-alfa therapy, the patient underwent a surgical procedure. To reduce the pronounced anterior projection of the vermillion, the patient underwent a transverse sickle-shaped mucosa en block removal between 1.2 and 1.5 cm dorsal to the vermillion border, basing on Conway's technique (Fig. 3). Intra- and extra oral mucosal flaps were harvested to isolate and remove all the edematous tissue, which involved part of the orbicularis muscle. Macroscopically infiltrated muscle was also removed. Once the area was completely remodeled, the excess of mucosa was removed and the defect was closed in a double layer. After 12-months follow-up, the patient did not show local recurrence of the disease and the oral continence was preserved, with normal lip sensation (Fig. 4).