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Management of ‘refractory’ skin disease in patients with lupus erythematosus

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Skin disease in patients with lupus erythematosus can be subdivided into two broad categories—those lesions that, when biopsied, demonstrate an interface dermatitis and those that do not demonstrate an interface dermatitis. The skin lesions that are represented by the interface dermatitis include discoid lupus erythematosus (DLE), subacute cutaneous lupus erythematosus (SCLE), and acute cutaneous lupus erythematosus. Many patients with these cutaneous lesions can be managed with ‘standard’ therapies, including sunscreens, protective clothing and behavioral alteration, and topical corticosteroids with or without an oral antimalarial agent. These standard therapies are often not used appropriately, resulting in a situation in which the patient is felt to have refractory disease. This chapter discusses these therapies and defines what is meant by refractory disease and how the author approaches these patients

Section snippets

Chronic cutaneous lupus erythematosus (CCLE)

CCLE can have several clinical manifestations. The most common subset is patients with DLE lesions. These patients might be classified as having localized DLE, when the lesions are only on the head and neck, or widespread DLE, when the lesions are on other body surfaces as well as the head and neck. Other, less common forms of chronic cutaneous LE include hypertrophic or verrucous (wart-like) lesions, lesions on the palms and/or soles, oral DLE, lupus tumidus, and lupus erythematosus

Subacute cutaneous lupus erythematosus (SCLE)

This subset is defined by the presence of skin lesions in which neither scarring nor atrophy occurs. In addition, follicular plugging is rarely observed in the lesions of SCLE. It is important to understand, however, that the patient in the SCLE subset can also have the scarring lesions of DLE, but these are not the predominant lesion. At least 50% of the patients with SCLE will have four or more features that classify them for a diagnosis of systemic LE. However, patients with SCLE differ from

Acute cutaneous lupus erythematosus (ACLE)

ACLE produces malar erythema, the classic ‘butterfly’ rash from which the term lupus erythematosus (wolf-like redness) was coined. The rash is induced by sun exposure or by exposure to other sources of ultraviolet light. Patients with a butterfly rash usually have active systemic disease, but there is no specific organ system involved.

Several cutaneous abnormalities had been part of the ACR criteria, but have been found to be less specific than photosensitivity. Diffuse hair loss (alopecia) was

Laboratory phenomena in patients with cutaneous LE

The full gamut of systemic disease manifestations of SLE can occur in patients with cutaneous disease, and thus these individuals can have any or all of the laboratory associations of the disorder. Serologic abnormalities are common in LE. They are more rare in patients with ‘pure’ cutaneous disease, such as DLE or HLE. The presence of abnormalities in these patients correlates with progressive disease or with the ACR criteria for SLE.7

The use of direct cutaneous immunofluorescence testing led

Management of cutaneous lupus erythematosus

Therapy of cutaneous lesions in patients with LE involves both an empiric as well as a scientific approach (Table 1).9, 10, 11, 12 Unfortunately, there are few double-blind, placebo-controlled trials of drugs utilized in the treatment of cutaneous LE. I generally approach patients with ‘specific’ lesions of LE in a similar manner when they have the relatively fixed lesions of subacute cutaneous LE (SCLE) (Figure 1) or chronic cutaneous LE manifest by discoid LE (DLE) lesions (Figure 2).

The

Conclusion

In summary, patients should be treated with sunscreens, protective clothing, behavioral alteration, topical corticosteroids, intralesional corticosteroids, and oral antimalarials as standard therapy. Attempts to reduce or stop smoking may aid in the control of cutaneous LE. The choice of alternative therapy is personal and discussions of the risks and benefits should be carefully documented. Successful therapy for cutaneous LE is possible in almost all well-motivated, cooperative patients.

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