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Oral Features of Mucocutaneous Disorders*

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Oral Features of Mucocutaneous Disorders* 1003.qxd 10/24/03 10:25 AM Page 1545 Academy Report Position Paper Oral Features of Mucocutaneous Disorders* Part of periodontology involves the diagnosis and treatment of a variety of non–plaque-related diseases of the periodontium. The International Workshop for a Classification of Periodontal Diseases and Conditions noted that the periodontist may be called upon to manage non–plaque-related mucocutaneous disorders either alone, or as part of a treatment team consisting of physicians, dentists or other allied health care professionals. This informational paper will review the etiology, clinical manifestations, diagnosis, and treatment of the most com- mon chronic mucocutaneous diseases, including those that may present as desquamative gingivitis or intra- oral vesiculobullous lesions. This paper is intended for the use of periodontists and other members of the dental profession. J Periodontol 2003;74:1545-1556. DESQUAMATIVE GINGIVITIS neous lesions occur in 0.4%.14 Ten percent to 20% of Desquamative gingivitis is a clinical feature of a vari- patients with lichen planus demonstrate oral as well ety of diseases. It is characterized by epithelial as cutaneous lesions.15 desquamation, erythema, ulceration, and/or the pres- Intraoral features of lichen planus include reticular, ence of vesiculobullous lesions of gingiva and other papular, plaque-like, atrophic, ulcerative, and bullous oral tissues. This phenomenon can be a manifesta- lesions. The reticular pattern occurs most frequently16 tion of a number of dermatoses, most commonly and is often seen as white lace-like lesions located lichen planus, cicatricial pemphigoid (benign mucous bilaterally on the buccal mucosa. The reticular, plaque- membrane pemphigoid), and pemphigus vulgaris1-3 like, and papular forms are generally asymptomatic (Tables 1 and 2). Biopsy specimens obtained from and may require no treatment. Patients with these types mucosal lesions may sometimes provide equivocal of lesions may report a change in surface texture or histopathologic findings and are often inadequate as roughness in the area that is affected. The atrophic, a single examination to establish the correct diagno- ulcerative, and bullous forms of the disease are referred sis because several diseases can produce a subep- to as erosive lichen planus. It is usually the onset of ero- ithelial blister. Therefore, direct immunofluorescence sive lesions that motivates patients to seek treatment. examination is necessary to establish a definitive Patients often present with a combination of painful diagnosis. Oral lesions may occur first or very early erosive lesions in conjunction with white lesions. in several mucocutaneous disorders.4-6 Accurate Patients with erosive lichen planus may exhibit desqua- diagnosis and effective treatment of these lesions mative gingivitis and a positive Nikolsky’s sign, char- may greatly diminish or reverse disease progression. acterized by epithelial separation from the underlying connective tissue as a result of minor trauma. A small LICHEN PLANUS percentage of patients with lichen planus will experi- Lichen planus is a relatively common dermatologic ence transient small bullae or vesicles involving the disease that affects the skin and mucous membranes, mucosal surfaces.17 including the oral cavity. Although the etiology of In addition to the oral cavity, lesions may also be lichen planus is unknown, its immunologic features seen on the skin, esophagus, genitalia, and rarely the suggest a cell-mediated immune response to intraep- eyes.18,19 Skin lesions occur alone or in combination ithelial antigens.7,8 Lichen planus generally develops with intraoral lesions and present as recurrent vio- between the ages of 40 and 70, and it is more com- laceus, keratotic, pruritic patches. Vulvovaginal-gingival mon in females than males.9,10 Skin and oral lesions and peno-gingival syndromes refer to a variant of of lichen planus in children are rare but have been lichen planus that affects the gingiva as well as the reported.11,12 Oral manifestations occur in approxi- genitourinary tract of either men or women.20,21 mately 2.0% of the general population,13 while cuta- In lichen planus, as well as other dermatologic dis- eases affecting the oral mucosa, biopsy specimens are * This paper was developed under the direction of the Research, Science and Therapy Committee and approved by the Board of Trustees of essential in establishing a diagnosis for erosive and the American Academy of Periodontology in May 2003. plaque-like forms and very helpful for reticular forms. J Periodontol • October 2003 1545 1003.qxd 10/24/03 10:25 AM Page 1546 Academy Report Ta b le 1. niques. In addition, cytoid bodies are com- Mucocutaneous Diseases That May Present monly seen at the epithelial-connective tis- sue interface and are thought to represent with Desquamative Gingivitis necrotic keratinocytes.27-29 Although the etiology remains elusive, these histologic Chronic ulcerative stomatitis Linear IgA disease Pemphigus and immunofluorescence features suggest Dermatitis herpetiformis Lupus erythematosus -Vulgaris that the condition represents a cell-mediated autoimmune response to basal keratino- Drug induced Pemphigoid - Vegetans cytes that express a foreign or altered self- 30,31 Epidermolysis bullosa aquisita - Bullous - Folliaceus antigen. This suggestion is supported by recent data which indicate that external Erythema multiforme - Cicatricial - Erythematosus substances such as mercury in dental amal- Graft-versus-host disease - Ocular - Paraneoplastic gams may induce keratinocyte ICAM-1 expression, increased binding of T cells to Lichen planus - Anti-epiligren - Benign famial normal keratinocytes, and increased pro- 32 Psoriasis duction of TNF-α in vitro. Lichenoid lesions resembling lichen planus may occur in association with the use of medications, including antimalarial drugs, anti- Ta b le 2. hypertensives, and non-steroidal anti-inflammatory Medications for the Treatment of Diseases agents.33 Lichenoid lesions demonstrate clinical, his- Associated with Desquamative Gingivitis tologic, and immunofluorescence patterns similar to idiopathic lichen planus, and they often resolve with- Topical Intralesional Systemic out recurrence following discontinuation of the iden- tified medication.34 Triamcinolone Triamcinolone Prednisone Exposure to dental restorative materials and cin- Fluocinonide Azathioprine namon flavoring agents has also been reported to induce lichenoid reactions.35-50 Lichen planus may Clobetasol Cyclophosphamide be associated with systemic diseases including hyper- Betamethasone Methotrexate tension and diabetes mellitus as well as hepatitis B and C.51-64 Lesions identical to lichen planus are seen in Halobetasol Gold patients with acute and chronic graft-versus-host dis- 65-72 Retinoids Dapsone ease and lupus erythematosus. Treatment of oral lichen planus requires elimination Tacrolimus Cyclosporin of potential factors associated with lichenoid reactions, elimination or control of local irritants, and the effec- tive use of therapeutic agents that suppress excessive The histologic features of lichen planus include epithe- lymphocyte function. Patients with erosive lichen planus lial acanthosis and hyperkeratosis, liquifaction degen- are often successfully treated with corticosteroids. eration of the epithelial basal cells, saw-tooth rete Topically applied medications such as fluocinonide and ridges, and a dense, band-like, sub-basilar infiltrate of clobetasol gel, beclomethasone dipropionate spray T lymphocytes.22-24 These classic histologic features (inhaler), or dexamethasone mouthrinses are effective are more commonly seen in skin biopsies, while in inducing remission of lesions.31,73-75 Short-term mucosal biopsy specimens are often less distinctive in tapering doses of systemic corticosteroids such as pred- character. Although immunofluorescence studies of nisone or intralesional injections are useful in severe lichen planus do not suggest pathognomonic features episodes as well as in recalcitrant cases.76 Although associated with the disease, direct immunofluorescence expensive to use, systemic and topically administered examination may be of value in supporting the diag- cyclosporin has shown promising results.77,78 Recently, nosis or ruling out other diseases.6,25,26 A linear or a topical tacrolimus has been shown to be an effective shaggy deposit of fibrin or fibrinogen at the basement form of treatment for oral lichen planus.79-83 Other membrane is often observed in biopsy specimens, which medications such as griseofulvin, azathioprine, cyclo- are examined using direct immunofluorescence tech- phosphamide, dapsone, retinoids, metronidazole, lev- 1546 Oral Features of Mucocutaneous Disorders Volume 74 • Number 10 1003.qxd 10/24/03 10:25 AM Page 1547 Academy Report amisole, thalidomide, and low molecular weight heparin bleeding surfaces beneath. Periods of exacerbation have shown some treatment efficacy, but evidence and remission are common, although some lesions based data are lacking. In addition, the potential for may remain unrelenting for years.117,118 The gingiva significant side effects may limit their use.84-94 Peri- is by far the most common intraoral site affected,116,119 odontists who administer these drugs should be aware and the lesions tend to heal with insignificant scarring. of reported side effects and be prepared to take appro- In contrast, ocular lesions often exhibit progressive priate action should any occur. A physician may need scarring leading to fusion of
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