Cheilitis glandularis (ORPHA:1221)
A rare skin disease of unknown origin characterized by macrocheilia and secretions of thick saliva from swollen labial minor salivary glands.
Epidemiology
Cheilitis glandularis (CG) prevalence is unknown. Less than a 100 cases have been reported to date, predominantly in adult males, and more rarely in adult females.
Clinical description
The clinical picture of CG consists of variable degrees of macrocheilia accompanied by the presence of red, dilated ostia of minor salivary glands on the vermilion area. A thick, mucoid material can be obtained from these ostia by manual expression. This viscous saliva often sticks to the vermilion causing discomfort to the patient. Hardening of superficial and deep tissues, including minor salivary glands, may occur. Rarely they may suppurate and drain a purulent discharge (cheilitis apostematosa profunda). Changes occur more frequently on the lower lip, but the upper lip is occasionally affected. Because of the macrocheilia, the vermilion is more exposed to ultra-violet radiation, which may lead to chronic actinic cheilitis and squamous cell carcinoma of the lower lip in rare cases.
Etiology
Cheilitis glandularis occurs more frequently in fair skinned adults and albino patients seem particularly prone, suggesting an influence of sunlight exposure. Nonetheless, the actual cause of CG remains unknown. Some consider CG as an inflammatory glandular disease while others think it merely represents a clinical reaction pattern to external trauma (mechanic, actinic) with no relation to salivary involvement as salivary glands have been found to be normal in some patients. Recently, changes in the immunohistochemical expression of water flow channels have been demonstrated in cases of CG.
Diagnostic methods
Diagnosis is mostly clinical. Histopathologic findings in the vermilion vary from spongiotic changes to chronic actinic epithelial damage, including superficial squamous cell carcinoma. Changes in minor salivary glands include chronic sialadenitis with dilated acinar lobules and ducts. Excretory ducts are ectatic and ductal metaplasia can be observed.
Differential diagnosis
Differential diagnosis may include cheilitis granulomatosa, cutaneous lupus erythematosus, angioedema, atopic cheilitis, actinic cheilitis, cheilitis artefacta, exfoliative cheilitis, ointment pseudo-cheilitis and benign and malignant minor salivary gland tumours (e.g. cystadenoma, cystadenocarcinoma and mucoepidermoid carcinoma).
Management and treatment
Mild cases do not require specific treatment. A lower lip vermilionectomy followed by careful dissection of the minor labial salivary glands is recommended for symptomatic and/or albino patients and is the only curative treatment for CG. Intralesional corticosteroids and oral antibiotics are ineffective. Long-term follow up is recommended, especially in very fair-skinned or albino patients, as the risk of labial squamous cell carcinoma is higher in these patients.
Prognosis
With proper treatment and surveillance, the prognosis is good.

                            Last update: 
                            August 2020


                            - Expert reviewer(s): 
                            Dr Marcello NICO