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| Gary M. White & Neil H. Cox |
| Diseases of the Skin |
20 |
Oral and Genital Disorders |
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Causes of cheilitis
Cheilitis is the term used to describe inflammation of the lips. Some specific morphologic patterns or causes are discussed in more detail here. The main causes and differential diagnoses are as follows.
| | Lip licking or lip chewing—these are most common in children (see later), but variants may occur in adults due to, for example, wetness related to playing a wind instrument. |
| | Allergic contact dermatitis—typical causes include medicaments applied to the lips, lipstick ingredients, sunscreen agents, toothpaste ingredients, colophony in dental floss and toothpicks, nail varnish, cosmetics, and nickel in the mouthpiece of a musical instrument. |
| | Atopic dermatitis—chronically dry lips are common. |
| | Actinic cheilitis—typically affects the lower lip in an adult with significant sun exposure history (see later). |
| | Angular cheilitis—a specific morphologic pattern discussed later. |
| | Orofacial granulomatosis and related conditions—see later. |
| | Idiopathic—some vitamins have been implicated in cheilitis (e.g.B12 and B2). |
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Figure 20.14 Lip licking. Only the skin accessible to the tongue is affected. |
Lip licking
Lip licking is an eczematous condition of the skin about the lips and represents an irritant contact dermatitis from the constant wetting and irritation by the saliva. Red, scaly, crusted, eczematous changes of the skin encircling the mouth in a child are characteristic (Fig.20.14). Only the skin accessible to the tongue is affected.
Children should be encouraged not to lick their lips. Petrolatum may be applied several times a day as a protectant. Occasionally, topical hydrocortisone (1% cream or ointment with or without an antiseptic additive) is helpful to decrease inflammation.
PRACTICE POINTS
| | Parents will often adamantly deny that their child licks or chews his or her lips, even though a few minutes’ observation is usually adequate to observe the proof. |
| | Allergic contact dermatitis can cause a persistently swollen lip. |
| | Ironically, lip salves used to treat allergic contact dermatitis of the lips may contain allergens that perpetuate it. |
Orofacial granulomatosis
Etiology and pathogenesis
In orofacial granulomatosis, the lips are persistently swollen, with granulomas seen histologically. When seen in conjunction with facial palsy and fissured (scrotal) tongue, the term Melkersson–Rosenthal syndrome is used. As part of their work-up, these patients should have patch testing and gastrointestinal evaluation. There is debate as to whether asymptomatic patients should undergo any further testing, for example endoscopic or radiographic evaluation, to exclude Crohn disease.
Clinical
The lips are enlarged in orofacial granulomatosis. In Melkersson–Rosenthal syndrome, sudden enlargement of one or both lips, which initially subsides but after multiple attacks persists, is characteristic (Fig. 20.15). A scrotal tongue is associated.
Differential diagnosis
Orofacial granulomatosis is a general term used when no associated conditions (listed here) are found. The following may produce the same morphologic picture.
| | Melkersson–Rosenthal syndrome—the lip swelling occurs in combination with a fissured (scrotal) tongue, facial palsy, and occasional swelling of some part of the face or scalp. Fever and headache may accompany the attacks. Onset is usually in the second decade. |
| | Allergic contact dermatitis—some patients may note worsening with certain foods. Allergy to potatoes, chocolate, cinnamic aldehyde, or monosodium glutamate has been found in some patients, and avoidance of such in the diet has led to improvement. Also, allergy to toothpaste and metals in crowns or fillings (e.g. gold and mercury) has caused orofacial granulomatosis. |
| | Cheilitis granulomatosis—a term used to describe Crohn disease affecting the lip. |
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Figure 20.15 Melkersson–Rosenthal syndrome. An enlarged lip (a) or lips and a fissured (scrotal) tongue (b) may occur. (c) Swelling may extend to the face. The cause is unknown. (Panels a and b courtesy of Michael O. Murphy, M.D.) |
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Figure 20.16 Candidal infection of the commissura. (Courtesy of G. Putnam.) |
Treatment
If an allergy to a food additive is found, dietary changes to avoid it should be made. If metal allergy is found, appropriate removal of fillings or crowns should be done. High-volume intralesional triamcinolone (3–10mL of 10mg/mL) after nerve block anesthesia has been recommended. Lip reduction surgery may be performed. Thalidomide, hydroxychloroquine, and clofazimine have been used.
Angular cheilitis
Etiology and pathogenesis
Redness, scaling, edema, and fissuring may occur at the corners of the mouth in angular cheilitis, also known as perlèche. This disease represents yet another type of intertrigo, where body folds keep the skin excessively moist and macerated. Saliva here causes skin irritation. Bacteria and Candida spp. that inhabit the mouth may cause secondary infection (Figs 20.16 and 20.17). Lip licking in the young, mouth breathing (day or night), or intraoral objects (e.g. expanders, braces, tongue studs, or dentures) causing excessive saliva are all risk factors. Decreased vertical separation of the mandible and maxilla (e.g. in older patients from lack of teeth, worn-down dentures, or bone resorption), combined with laxity of facial skin, causes a prominent skin fold. Oral examination should exclude intraoral candidiasis. Rarely, it may be due to iron deficiency.
Clinical
Redness, scaling, and crusting at the corner of the mouth occur in angular cheilitis (Fig. 20.17).
Differential diagnosis
There is no differential diagnosis as such, as the term describes a pattern of cheilitis; the differential diagnosis is between the various contributing causes discussed here, more than one of which may be relevant.
Treatment
All the causes listed here should be eliminated as far as possible; in particular, old dentures should be replaced. A low- to medium-potency topical steroid with added imidazole or nystatin is usually effective treatment, but the condition will recur unless the cause is addressed. Petrolatum or other ointments to protect the skin may help; a topical antibiotic (e.g. mupirocin three times a day) may be appropriate if microbiology swabs show that there is also staphylococcal infection. Excessive skin folding may also be corrected by collagen injection.
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Figure 20.17 (a,b) Angular cheilitis. This intertrigo of the folds of the lip is common in older patients, who develop a deep fold. Bacteria and Candida may superinfect. |
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Figure 20.18 Superficial mucocele. |
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Figure 20.19 Lip fissure. This responded to a topical steroid–antibiotic preparation. |
PRACTICE POINTS
| | Angular cheilitis is usually an irritant contact dermatitis from excessive saliva. Either yeasts or bacteria can cause secondary infection. |
| | The commonest cause in older adults is decreased vertical separation of the mandible and maxilla due to worn-down dentures or bone resorption, combined with increased facial skin laxity. |
| | Iron deficiency is often cited as a likely cause of angular cheilitis but is far less common than the facial shape changes discussed; even if it coexists, angular cheilitis rarely responds to iron supplementation. |
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Figure 20.20 Cheilitis glandularis. (a) The lower lip is enlarged and the surface may be rough. Small beads of viscous fluid may be extruded with pressure. (b) A string of mucus may be formed between the lip and examining finger. |
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Figure 20.21 Fordyce spots. |
Superficial mucocele
Superficial mucoceles are subepithelial extravasations of sialomucin that occur at the epithelial–connective tissue interface and are directly related to minor salivary glands. They have been associated with trauma, oral lichen planus, and oral lichenoid graft-versus-host disease. Asymptomatic, small, clear, tense vesicles with a predilection for the retromolar pad area, posterior buccal mucosa, and soft palate are characteristic (Fig.20.18). No treatment is needed. Spontaneous resolution occurs.
Lip fissure
A child, typically aged 7 or 8 years, may develop a painful and persistent fissure of the lip (Fig.20.19). Conditions that are thought to predispose to this lesion are mouth breathing, sun, and wind. A topical steroid with antibiotic (e.g. tetracycline) or antiseptic additives should be tried. Rarely, adults may develop a chronic lip fissure secondary to irritation from contact with misaligned anterior teeth.
Cheilitis glandularis
Swelling of the lower lip (Fig.20.20a) with multiple vesicles, or the formation of beads of clear fluid with gentle pressure, is characteristic. Some believe that three types of lip damage may cause cheilitis glandularis, namely actinic, atopic, and factitial. SCC may develop, presumably because of the increased UV exposure. The string sign is the formation of a string of mucus between the finger and lip (Fig. 20.20b). If the underlying cause is known, treatment should be given. Mild cases may simply be observed only. In severe cases, macrocheiloplasty has been performed.
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Figure 20.22 Leukoplakia of the lower lip, a typical site in a chronic pipe smoker. |
Fordyce spots
Multiple, pinpoint, yellow papules on the lips or buccal mucosa are characteristic. They represent ectopic sebaceous glands and are usually an incidental finding requiring no treatment. They have been reported to disappear during isotretinoin therapy and then reappear after the drug is discontinued (Fig. 20.21).
Actinic cheilitis and leukoplakia of the lip
Chronic UV damage to the lower lip can cause diffuse actinic damage (actinic cheilitis, see Figs 32.11–32.13) or localized lesions of leukoplakia. A white hyperkeratotic plaque with a sharply defined proximal edge on the lower lip of a man with a history of chronic sun exposure is characteristic (Fig.20.22). The biopsy may remove the lesion. Otherwise, cryotherapy may be done. If the lesion does not completely resolve with cryotherapy, biopsy to exclude SCC is mandatory. In cases where much of the lower lip is involved, vermilionectomy with a mucosal advancement flap (‘lip shave') was sometimes performed but has largely been replaced by laser shaving, for example with a CO2 laser.
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Figure 20.23 Squamous cell carcinoma of the oral mucosa. (a) Leukoplakia.(b) Invasive carcinoma of the floor of the mouth. (c) Invasive carcinoma of the tongue. (Courtesy of G. Putnam.) |
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Figure 20.24 (a,b) Laugier–Hunziker syndrome: benign lentigines of the oral mucosa are shown here. Longitudinal melanonychia and pigmented macules of the digits occur as well (see Fig. 29.44). |
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Figure 20.25 Disappearing vermilion. |
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Figure 20.26 Drug-induced hypertrophy of the gums. |
Squamous cell carcinoma of the oral mucosa
Squamous cell carcinoma may occur inside the mouth. A history of both smoking and drinking are predisposing factors to the development of SCC of the oral mucosa. Early lesions are in situ. Lesions inside the mouth may be either white (leukoplakia, Fig.20.23a,b) or red (erythroplakia, Fig.20.23c). Both are fixed in location and slowly enlarge and thicken over time. Excision with appropriate margins by a maxillofacial specialist is indicated.
Squamous cell carcinoma of the lip
Squamous cell carcinoma accounts for the vast majority of all lip cancers, almost all of these occurring on the lower lip because of its exposure to the sun (basal cell carcinoma occurs on the skin of the upper lip but uncommonly abuts on to the vermilion). A slowly enlarging, hyperkeratotic papulonodule originating from the vermilion border of the lower lip is characteristic. SCC of the lip has a higher incidence of metastasis (3–20%) compared with cutaneous SCC. Wedge resection is the preferred treatment. Mohs surgery is also highly effective for both primary and recurrent SCC, and some have recommended it as the treatment of choice. Treatment of coexisting actinic cheilitis (see earlier) may lower the risk of recurrence.
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Figure 20.27 Black hairy tongue. |
Laugier–Hunziker syndrome
Patients with Laugier–Hunziker syndrome develop pigmented macules of the lips, oral mucosa, and/or the nail apparatus (e.g. longitudinal melanonychia, Fig.20.24) in the absence of systemic disease. Other sites, such as the fingertips, palms, feet, esophagus, and vulva, have been reported to be involved. The lesions may be brown-black or bluish black. Onset is in adult life, with women affected more commonly than men. Familial cases have been reported. Only oral pigmentation may occur, or both oral and nail pigmentation. Pale-skinned people are affected almost exclusively. An upper gastrointestinal tract barium meal or endoscopy can help exclude the polyposis associated with Peutz–Jeghers syndrome, but this is never really a consideration due to the totally different age group affected, plus the facial lentiginosis and the gastrointestinal features of Peutz–Jeghers syndrome. Histology shows increased pigmentation in the basal layers, along with pigmentary incontinence. No treatment is needed, but the laser may remove cosmetically unsightly lesions.
Fixed drug eruption
The fixed drug eruption (see Ch.18) may also occur in the mouth. A tender erosive lesion is seen.
Disappearing vermilion
In this benign disorder, there is a slowly increasing area of pallor (but normal texture) at the junction of the skin and vermilion. It is relatively common and more frequent in women (Fig. 20.25).
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White/Cox: Diseases of the Skin, 2ed.(c) 2006, Elsevier Inc. All rights reserved.