Chapter 055. Immunologically Mediated Skin Diseases (Part 9)

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Discoid lupus erythematosus. Violaceous, hyperpigmented, atrophic plaques, often with evidence of follicular plugging, which may result in scarring, are characteristic of discoid lupus erythematosus (also called chronic cutaneous lupus erythematosus). Scleroderma and Morphea The skin changes of scleroderma (Chap. 316) usually begin on the hands, feet, and face, with episodes of recurrent nonpitting edema. Sclerosis of the skin begins distally on the fingers (sclerodactyly) and spreads proximally, usually accompanied by resorption of bone of the fingertips, which may have punched out ulcers, stellate scars, or areas of hemorrhage (Fig. 55-7). The fingers may actually shrink in size and become sausage-shaped, and since...

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Chapter 055. Immunologically Mediated Skin Diseases (Part 9) Figure 55-6
Discoid lupus erythematosus. Violaceous, hyperpigmented, atrophic plaques, often with evidence of follicular plugging, which may result in scarring, are characteristic of discoid lupus erythematosus (also called chronic cutaneous lupus erythematosus). Scleroderma and Morphea The skin changes of scleroderma (Chap. 316) usually begin on the hands, feet, and face, with episodes of recurrent nonpitting edema. Sclerosis of the skin begins distally on the fingers (sclerodactyly) and spreads proximally, usually accompanied by resorption of bone of the fingertips, which may have punched out ulcers, stellate scars, or areas of hemorrhage (Fig. 55-7). The fingers may actually shrink in size and become sausage-shaped, and since the fingernails are usually unaffected, the nails may curve over the end of the fingertips. Periungual telangiectases are usually present, but periungual erythema is rare. In advanced cases, the extremities show contractures and calcinosis cutis. Facial involvement includes a smooth, unwrinkled brow, taut skin over the nose, shrinkage of tissue around the mouth, and perioral radial furrowing (Fig. 55-8). Matlike telangiectases are often present, particularly on the face and hands. Involved skin feels indurated, smooth, and bound to underlying structures; hyperpigmentation and hypopigmentation are also often present. Raynaud's
phenomenon, i.e., cold-induced blanching, cyanosis, and reactive hyperemia, is present in almost all patients and can precede development of scleroderma by many years. The combination of calcinosis cutis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia has been termed the CREST syndrome. Anticentromere antibodies have been reported in a very high percentage of patients with the CREST syndrome but in only a small minority of patients with scleroderma. Skin biopsy reveals thickening of the dermis and homogenization of collagen bundles. Direct immunofluorescence microscopy of lesional skin is usually negative. Figure 55-7
Scleroderma showing acral sclerosis and focal digital ulcers. Figure 55-8