Abstract
This series of three papers reviews the causes, diagnosis and differential diagnosis, and outlines the management of sore and/or swollen lips.
From Volume 44, Issue 1, January 2017 | Pages 70-74
This series of three papers reviews the causes, diagnosis and differential diagnosis, and outlines the management of sore and/or swollen lips.
Lesions on the lips can be disfiguring and of serious aesthetic concern to patients, and may herald usually local or sometimes systemic diseases. Most important for the clinician is not only to give an early diagnosis, providing an accurate treatment, but mainly to distinguish which lesions are malignant or potentially malignant.
The diagnosis of sore/swollen lips is based mainly on:
A history of exposure to violence, caustic foods/liquids, wind or solar radiation or cosmetic and food allergens, drugs, social and travel history, close contact with patients or animals infected with various microbial agents, can help the diagnosis.
A lesion's location and clinical characteristics are very useful tools for the diagnosis. The location of some cheilitis is unique, the commissures, for example, are the characteristic involvement sites for angular cheilitis.1 Other findings, such as a widespread exfoliation, a characteristic of exfoliative cheilitis,2 or a mucopurulent discharge of glandular cheilitis may help.3,4 The presence of similar lesions in the mouth and lips are characteristics of plasma cell cheilitis5 and lesions on the skin and other mucosae are usually seen in atopic6 and actinic prurigo cheilitis.7
The clinical diagnosis may need to be confirmed by laboratory findings, such as a positive culture in infective cheilitis,8 a patch test in contact allergic and granulomatous cheilitis,9 or histology and immunology in glandular cheilitis.10
The most important histological pathognomonic characteristics in cheilitis are:
The differential diagnosis involves other cheilitis and local or systemic diseases with common manifestations of sore or swollen lips. For example, angular cheilitis must be excluded from herpetic cheilitis. Both conditions can arise when the immune system is weak.8,21,22 Both cause physical discomfort and pain and require immediate treatment.23 However, the cause is different as bacteria like Staphylococcus or Streptococcus and fungus like Candida albicans are responsible for the angular cheilitis, while viruses like HSV-1 or 2 are for the herpetic cheilitis. Angular cheilitis starts in the commissures as dry, red skin which soon exfoliates, while the herpetic cheilitis starts with a tingling/itching sensation all over the lips followed by numerous, discrete small vesicles which break easily leaving painful erosions. These erosions are contagious but heal within 2–3 weeks, while the lesions in angular cheilitis are chronic and rarely passed on by close contact.
Although laboratory investigations (haematologic, biochemical and immunological blood tests; microscopy; cultures and more advanced PCR techniques) can help differentiate these diseases,24,25 their diagnosis is based mainly on their clinical characteristics.
The wide extension of lip cracking and a recent patient's history of exposure in wind or sun allow the diagnosis of the simplex (factitious) cheilitis from the angular cheilitis. Some patients with cracked lips show additionally excessive production of keratin in the vermillion border, a characteristic finding in exfoliative cheilitis.26
In the differential diagnosis of actinic cheilitis, other malignant, pre-malignant, metastatic, inflammatory and eczematoid or photo-sensitive disorders (like follicular cheilitis27), irritant contact or allergic and granulomatous cheilitis are included.
The diagnosis must be based not only on the evolution time, the history of recent sunlight exposure and the clinical course, but also on the histological findings from lip or skin biopsies.28
In the differential diagnosis of chronic lip swelling, glandular cheilitis29 and other causes such as actinic,30 granulomatous3 or exfoliative cheilitis,31 multiple mucoceles32, cystadenocarcinoma and mucoepidermoid carcinoma are included.33
Management should be focused on:34
The elimination of underlying predisposing factors is often helped by preventing the following:
Lip dryness and cracking can be minimized by the patient by:
The prevention of various infections in the lips can be achieved by:
Identification and confirmation of the causative allergens of cheilitis by patch tests9 and their exclusion by using special diets free of cinnamon and benzoate products, or by replacing any suspicious drugs,35,36 are the necessary treatment steps in some allergic, granulomatous and drug-induced cheilitis.
Relief from discomfort can be achieved with the use of anti-inflammatory medicines and from itching and erythema with the use of anti-histamine drugs and corticosteroids locally (in creams or injections) or systematically. Antibacterial and antifungal creams are recommended for angular cheilitis while antiviral drugs, mostly creams, alone or with corticosteroids, are given for herpetic cheilitis.37
Antibiotics in creams, tablets or injections may prevent secondary infection which can be a serious complication of trauma, infective, glandular and other forms of cheilitis where the patient‘s immune status is impaired.
In cheilitis simplex, the general protective measures of proper clothing, including wide-brimmed hats, lip emollients and the use of menthol or camphor, alone may relieve the discomfort.33 In exfoliative cheilitis, additionally, a combination of corticosteroids and antibiotics, or tacrolimus, twice a day, may ameliorate the symptoms and exfoliation,31 while the systemic use of prednisolone (10–15 mg/day) for 2–3 weeks with or without vitamin A, C, D or B complex38 may help in severe cases. Antidepressants and laser applications have also been effective in some patients.39
The first choice of local treatment for granulomatous cheilitis is the intralesional injection of corticosteroids such as of triamicinolone acetone, under local anaesthesia, 3–6 injections in a 6-month period or, rarely, lip plastic surgery,3 which is better given in secondary care. Recurrence and aesthetic problems are the main complications of local treatment. Thalidomide and dapsone or clofazimine20 are the second choices of systemic treatment, while systemic corticosteroids, such as prednisolone (up to 40 mg, initial daily dose) together with antibiotics like minocycline 100–200 mg per day for 4–6 months may be the treatment of choice. Topical corticosteroids, tacrolimus 0.03%, griseofulvin or ciclosporin, show promising results in plasma cell cheilitis but systemic corticosteroids remain the treatment of choice.19,20
Vermillionectomy (the surgical removal of the vermillion border of lips) is recommended for the treatment of glandular cheilitis29,40 or intralesional injections of steroids, together with broad spectrum antibiotics like minocycline or tacrolimus.4
In angular cheilitis, for example, the treatment of any deficiency state or of anaemia is a fundamental step. Local measures must focus, firstly, in improving oral and dental appliance hygiene. Dental appliances must be properly designed and cleaned at regular intervals with detergents and disinfectants such as hypochlorite.25 The patient should apply a commissures cream containing an antifungal (fluconazole, nystatin, amphotericin or miconazole)41 or antibiotic such as fusidic acid.42 In recalcitrant lesions, corticosteroids alone or in combination with antifungals for two weeks may help.
Most cheilitis are benign but the malignant potential of some forms of them (actinic, glandular and discoid lupus erythematosus, or in patients with a cancer predisposition) is of serious concern. Untreated grandular and chronic actinic cheilitis cases develop lip carcinoma in up to 3.5–38.5% of neglected cases in a long follow-up period.43
The histological findings and especially the presence or absence of dysplasia and its severity determines the treatment plan for actinic cheilitis. Acute and chronic actinic cheilitis without dysplasia require, simply, prophylactic measures such as lip protection by block balms, moisturizing petroleum-based lipgloss and appropriate hats and clothing.44 Local application of trichloracetic acid, 5 fluorouracil or imiquimod, twice daily, has often been used with some success, despite the long treatment required (up to 6 weeks) and the adverse effects of a peri-oral skin irritation. Newer therapies, such as photodynamic, laser or cryotherapy may have better results with minimal complications.28,45,46,47 In cases of severe dysplasia or carcinoma in situ, the treatment must be more aggressive, such as surgical excision, vermillionectomy, or even brachytherapy.27
This series of three papers has reviewed the causes, diagnosis and differential diagnosis, and outlined the management, of sore and/or swollen lips. Some lip diseases last a few days and some others remain for months or years causing severe patient concern. Some are benign but others need extra care due to their increased risk for malignant transformation. Clinicians should know briefly about the diseases which cause sore and/or swollen lips as summarized in Figure 1.