LUPUS ERYTHEMATOSUS (SYSTEMIC AND DISCOID)
Clinical Features, Diagnosis, and Treatment
Clinical Features, Diagnosis, and Treatment
Systemic lupus erythematosus (SLE) is a prototypical example of an immunologically mediated inflammatory condition that causes multiorgan damage. (SLE is discussed in more detail in Chapter 18, under “Connective Tissue Diseases”; the brief description here emphasizes its clinical manifestations on the oral mucosa.)
The oral lesions of systemic lupus are generally similar to those of discoid lupus (see below) and are most prevalent on the buccal mucosa, followed by the gingival tissues, the vermilion border of the lip, and the palate, in decreasing order of frequency. The lesions are frequently symptomatic, especially if the patient ingests hot or spicy foods, and often consist of one or more of the following components: erythema, surface ulceration, keratotic plaques, and white striae or papules (Figure 5-37). These lesions frequently appear lichenoid although they may be nonspecific and resemble leukoplakia, vesiculobullous disease, or even a granulomatous lesion (Figure 5-38). They typically respond well to topical or systemic steroids. Clobetasol (a potent topical steroid) placed under an occlusive tray is very effective for temporary relief of these lesions.Long-term remission of these lesions obviously depends on treatment of the underlying systemic disease.
Discoid lupus erythematosus (DLE) is a relatively common disease and occurs predominantly in females in the third or fourth decade of life.DLE can present in both localized and disseminated forms and is also called chronic cutaneous lupus (CCL). DLE is confined to the skin and oral mucous membranes and has a better prognosis than SLE.
Typical cutaneous lesions appear as red and somewhat scaly patches that favor sun-exposed areas such as the face, chest, back, and extremities. These lesions characteristically expand by peripheral extension and are usually disk-shaped. The oral lesions can occur in the absence of skin lesions, but there is a strong association between the two. As the lesions expand peripherally, there is central atrophy, scar formation, and occasional loss of surface pigmentation. Lesions often heal in one area only to occur in a different area later.
The oral mucosal lesions of DLE frequently resemble reticular or erosive lichen planus. The primary locations for these lesions include the buccal mucosa, palate, tongue, and vermilion border of the lips. Unlike lichen planus, the distribution of DLE lesions is usually asymmetric, and the peripheral striae are much more subtle (Figure 5-39). The lesions may be atrophic, erythematous, and/or ulcerated and are often painful. Hyperkeratotic lichen planus–like plaques are probably twice as common in patients with CCL as compared to patients with SLE.The oral lesions of DLE are markedly variable and can also simulate leukoplakia. Therefore, the diagnosis must be based not only on the clinical appearance of the lesions but also on the coexistence of skin lesions and on the results of both histologic examination and direct immunofluorescence testing. Despite their similar clinical features, lichen planus and lupus erythematosus yield markedly different immunofluorescent findings. Some authors
believe that the histology of oral lupus erythematosus is characteristic enough to provide a definitive diagnosis at the level of light microscopy, but most feel that the diagnostic standard must involve direct immunofluorescence. Importantly, lesions
Typical cutaneous lesions appear as red and somewhat scaly patches that favor sun-exposed areas such as the face, chest, back, and extremities. These lesions characteristically expand by peripheral extension and are usually disk-shaped. The oral lesions can occur in the absence of skin lesions, but there is a strong association between the two. As the lesions expand peripherally, there is central atrophy, scar formation, and occasional loss of surface pigmentation. Lesions often heal in one area only to occur in a different area later.
The oral mucosal lesions of DLE frequently resemble reticular or erosive lichen planus. The primary locations for these lesions include the buccal mucosa, palate, tongue, and vermilion border of the lips. Unlike lichen planus, the distribution of DLE lesions is usually asymmetric, and the peripheral striae are much more subtle (Figure 5-39). The lesions may be atrophic, erythematous, and/or ulcerated and are often painful. Hyperkeratotic lichen planus–like plaques are probably twice as common in patients with CCL as compared to patients with SLE.The oral lesions of DLE are markedly variable and can also simulate leukoplakia. Therefore, the diagnosis must be based not only on the clinical appearance of the lesions but also on the coexistence of skin lesions and on the results of both histologic examination and direct immunofluorescence testing. Despite their similar clinical features, lichen planus and lupus erythematosus yield markedly different immunofluorescent findings. Some authors
believe that the histology of oral lupus erythematosus is characteristic enough to provide a definitive diagnosis at the level of light microscopy, but most feel that the diagnostic standard must involve direct immunofluorescence. Importantly, lesions
FIGURE 5-38 Lupus lesions frequently appear lichenoid but may be nonspecific and resemble leukoplakia.
with clinical and immunofluorescent features of both lichen planus and lupus erythematosus have also been described (overlap syndrome).
with clinical and immunofluorescent features of both lichen planus and lupus erythematosus have also been described (overlap syndrome).
Histopathologic Features
The histopathologic changes of oral lupus consist of hyperorthokeratosis with keratotic plugs, atrophy of the rete ridges, and (most especially) liquefactive degeneration of the basal cell layer. Edema of the superficial lamina propria is also quite prominent. Most of the time, lupus patients lack the bandlike leukocytic inflammatory infiltrate seen in patients with lichen planus. Immediately subjacent to the surface epithelium is a band of PAS-positive material, and frequently there is a pronounced vasculitis in both superficial and deep connective tissue. Another important finding in lupus is that direct immunofluorescence testing of lesional tissue shows the deposition of various immunoglobulins and C3 in a granular band
The histopathologic changes of oral lupus consist of hyperorthokeratosis with keratotic plugs, atrophy of the rete ridges, and (most especially) liquefactive degeneration of the basal cell layer. Edema of the superficial lamina propria is also quite prominent. Most of the time, lupus patients lack the bandlike leukocytic inflammatory infiltrate seen in patients with lichen planus. Immediately subjacent to the surface epithelium is a band of PAS-positive material, and frequently there is a pronounced vasculitis in both superficial and deep connective tissue. Another important finding in lupus is that direct immunofluorescence testing of lesional tissue shows the deposition of various immunoglobulins and C3 in a granular band
involving the basement membrane zone. Importantly, direct immunofluorescent testing of uninvolved skin in a case of SLE will show a similar deposition of immunoglobulins and/or complement. This is called the positive lupus band test, and discoid lesions will not show this result.
Malignant Potential, Importance, and Scope of Oral Lesions
The precancerous potential of intraoral discoid lupus is controversial. Basal cell and (more commonly) squamous cell carcinomas have been reported to develop in healing scars of discoid lupus. However, this malignant change may have been caused by the radiation and ultraviolet light used in the treatment of lupus in the early twentieth century. The development of squamous cell carcinoma has been described in lesions of discoid lupus involving the vermilion border of the lip, and actinic radiation may play an important adjunct role in this.
Oral ulcers are one of the defining features of lupus erythematosus. The frequency of oral lesions in all forms of lupus combined varies from 5 to > 50%. Importantly, oral ulcers are found in SLE patients who have a higher level of disease activity as measured by the system lupus activity measure.
One study of 446 SLE patients showed that the extent of oral mucosal involvement was 40 to 54%, with the higher figure occurring in more severely involved patients.The validity of these percentages, however, may be in question. In a recent survey of international centers devoted to the treatment of lupus, there was an extremely low level of agreement on the incidence of oral manifestations of this disease.An increase in the frequency of generalized periodontal disease has been reported with SLE.However, most studies have found that there is either a decrease in periodontal probing depth in lupus or no change in periodontal status.
In fact, recent evidence suggests that the tendency of periodontitis to be more severe or progressive in some patients with collagen vascular diseases is a consequence of xerostomia and not a result of the primary disease.
Malignant Potential, Importance, and Scope of Oral Lesions
The precancerous potential of intraoral discoid lupus is controversial. Basal cell and (more commonly) squamous cell carcinomas have been reported to develop in healing scars of discoid lupus. However, this malignant change may have been caused by the radiation and ultraviolet light used in the treatment of lupus in the early twentieth century. The development of squamous cell carcinoma has been described in lesions of discoid lupus involving the vermilion border of the lip, and actinic radiation may play an important adjunct role in this.
Oral ulcers are one of the defining features of lupus erythematosus. The frequency of oral lesions in all forms of lupus combined varies from 5 to > 50%. Importantly, oral ulcers are found in SLE patients who have a higher level of disease activity as measured by the system lupus activity measure.
One study of 446 SLE patients showed that the extent of oral mucosal involvement was 40 to 54%, with the higher figure occurring in more severely involved patients.The validity of these percentages, however, may be in question. In a recent survey of international centers devoted to the treatment of lupus, there was an extremely low level of agreement on the incidence of oral manifestations of this disease.An increase in the frequency of generalized periodontal disease has been reported with SLE.However, most studies have found that there is either a decrease in periodontal probing depth in lupus or no change in periodontal status.
In fact, recent evidence suggests that the tendency of periodontitis to be more severe or progressive in some patients with collagen vascular diseases is a consequence of xerostomia and not a result of the primary disease.