Chronic Cutaneous Lupus Erythematosus Discoid Lupus Erythematosus

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Discoid LE (DLE) is the most common form of LE. Clinically, the head and neck region is affected in most cases. On the face, there may be a butterfly distribution. However, in some cases, the trunk and upper extremities can be also involved. Lesions consist of erythematous scaly patches and plaques.

Histologically, in DLE the epidermis and dermis are affected, and the subcutaneous tissue is usually spared. However, patchy infiltrates may be present. Characteristic microscopic features are hyperkeratosis with follicular plugging, thinning, and flattening of the epithelium and hydropic degeneration of the basal layer (liquefaction degeneration) (Fig. 21.1). In addition, there are scattered apoptotic keratinocytes (Civatte bodies) in the basal layer or in the epithelium. Particularly in older lesions, thickening of the basement membrane becomes obvious in the periodic acid-Schiff stain. In the dermis, there is a lichenoid or patchy lymphocytic infiltrate with accentuation of the pilosebaceous follicles. There is interstitial mucin deposition and edema, and usually no eosinophils and neutrophils are present.

Hypertrophic lesions have acanthosis and hyperkeratosis of the epidermis (Wee-don 2002). Direct immunofluorescence is an important test that should be performed in this subtype. Usually, there is deposition of IgG and IgM in 50%-90% of cases. The differential diagnosis includes drug reaction, dermatomyositis, graft-vs-host disease, and mycosis fungoides. Jessner's lymphocytic infiltration of the skin usually does not involve the epidermis as hydropic degeneration of the basal layer and scattered apop-totic keratinocytes do not occur. Otherwise, the dermal infiltrate is identical to LE. Consequently, Jessner's lymphocytic infiltration of the skin is regarded as a variant of LE by some authors. In a drug reaction there are frequently eosinophils that are not

Fig. 21.1. Discoid lupus erythematosus. Hydropic degeneration of the basal layer

Fig. 21.1. Discoid lupus erythematosus. Hydropic degeneration of the basal layer

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seen in DLE. In graft-vs-host disease, there is usually not a prominent lymphocytic infiltrate involving the dermis. Early-stage dermatomyositis can be histologically difficult to distinguish from DLE because it can exhibit identical changes. Consequently, clinical features should be considered for diagnosis. In mycosis fungoides, lymphocytes frequently exhibit nuclear atypia.

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