Chronic Vesiculoulcerative Disease

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PEMPHIGUS VULGARIS Pemphigus vulgaris is a rare disease with an incidence of 0.5 to 3.2 per 100,000 persons annually. It is important, though, because without treatment, its mortality rate is high. Oral manifestations occur in up to 70 percent of patients and are frequently the first sign of disease, preceding cutaneous lesions by up to a year. Oral lesions are also the most difficult to treat. Ulceration results from autoantibodies attacking the deeper layer of the stratum spinosum of the epidermis, resulting in an intraepithelial split. Females are affected slightly more commonly, with a female-to-male ratio of 2:3. Pemphigus vulgaris most commonly affects people in their sixth decade of life. Typical lesions are superficial erosions with ragged edges found on any oral mucosal surface. Involvement of the soft palate is most common, occurring 80 percent of the time. The buccal mucosa is the next most common site of involvement (46 percent), followed by the tongue (20 percent). Cutaneous lesions appear as fluid-filled vesicles and bullae. Lesions can be induced by firm lateral pressure on apparently normal skin (Nikolsky's sign). Treatment includes systemic corticosteroids as well as other immunosuppressive agents and is beyond the realm of emergency medicine. Referral to an appropriate consultant is necessary.1 49

CICATRICIAL PEMPHIGOID Cicatricial pemphigoid is a chronic vesiculobullous mucocutaneous disorder with an autoimmune etiology. Autoantibodies against the basement membrane result in subepithelial cleft formation. Cicatricial pemphigoid affects people in the seventh decade of life, with no race predilection. The ratio of males to females 1:2.27. Oral lesions are seen in approximately 85 percent of patients, but any mucosal or cutaneous site can be affected. Oral lesions may begin as a desquamative gingivitis or vesiculobullous lesions. Oral lesions eventually become denuded, leaving a painful, erythematous, irregularly bordered erosion or ulcer. Lesions may persist for weeks. Significantly, ocular involvement occurs in 65 percent of cicatricial pemphigoid patients and, if untreated, may lead to blindness. Referral to an ophthalmologist is essential. Diagnosis is by biopsy and immunofluorescent staining of perilesional mucosa. Histologically, the lesions show inflammatory infiltrates surrounding subepithelial bullae. Treatment must be individualized. Treatment of isolated oral lesions with topical steroids is acceptable. Intralesional steroid injections may be useful. Systemic involvement or persistent oral lesions require systemic steroid therapy. 1 50

ERYTHEMA MULTIFORME Erythema multiforme most commonly affects men in the third or fourth decade of life. Early cutaneous lesions are concentric erythematous circular rings resembling a target (target lesion). Oral lesions are painful erythematous patches that undergo necrosis, forming large ulcerations with irregular borders. They occur most commonly on the lips, labial mucosa, buccal mucosa, tongue, floor of the mouth, and soft palate, sparing the hard palate and gingiva. Erythema multiforme is a spectrum of diseases that includes toxic epidermal necrosis in its most severe form. Stevens-Johnson syndrome includes ocular and genital involvement accompanying oral lesions. Treatment is with systemic steroids in all patients except those with isolated oral lesions. In such patients, topical steroids may be adequate.1651

LICHEN PLANUS Lichen planus is one of a group of chronic cutaneous vesiculoerosive diseases affecting approximately 1 percent of the population. Females are affected three times as often as males. Certain drugs such as ACE inhibitors have been shown to cause lichenoid-type reactions, and these must be differentiated from lichen planus. Lichen planus is a result of T-lymphocyte attack on the basal cell layer. Approximately, 50 percent of patients with cutaneous lesions have oral lesions, but only 25 percent of patients have isolated oral lesions. Lichen planus can affect any mucosal surface; however, orally, the buccal mucosa is most common (Fig 234-14). In its reticular form, lichen planus appears as multiple scattered white papules interconnected via white lines called Wickham's striae. Definitive diagnosis is via biopsy and immunofluorescent staining. The reticular and papular patterns are usually asymptomatic and require no treatment. Symptomatic erosive and atrophic forms of lichen planus, especially those involving the tongue, can be treated successfully with topical corticosteroids. Some question concerning the premalignant potential of lichen planus, especially the erosive form, exists, but less than 1 percent of oral lichen planus undergoes malignant transformation. 1652

Graft-versus-Host Disease

Graft-versus-host disease occurs in up to 50 percent of allogeneic bone marrow transplants despite HLA matching and immunosuppressive therapy. In graft-versus-host disease, the transplanted bone marrow cells recognize the host as foreign, and transplanted hematopoetic cells attack the host. Systemic manifestations of the disease vary by the severity of disease, organ system affected, and acute versus chronic disease. Oral manifestations include lesions in a fine white papular pattern, interlacing white striae in a lichenoid or reticular pattern, and a desquamative pattern occurring commonly on the tongue and labial and buccal mucosa. Posttransplant ulcerations related to the induced neutropenic state occur in the first 2 weeks. Ulcerations that persist longer must be attributed to graft-versus-host disease. Xerostomia and a burning oral sensation are common. The oral lesions and xerostomia as a result of chronic graft-versus-host disease may persist for years. An increased incidence of periodontal disease and dental caries is associated with decreased salivary flow. The principal approach to treatment is to prevent disease with the addition of other immunosuppressive agents. Oral topical steroids improve healing of oral ulceration and psoralen and ultraviolet A (PUVA) therapy may be used on cutaneous oral lesions.4 53

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