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Figure 15-1 illustrates cutaneous involvement of systemic lupus erythematosus (SLE) in the classic butterfly pattern on the face. This macular and intensely erythematous eruption is frequently aggravated by sun exposure and may flare with other symptoms of systemic disease. Figure 15-2 shows the temporary alopecia that is also a hallmark of SLE. This autoimmune disease of unknown etiology affects almost every organ system. The most common findings in the child with SLE are fever, arthralgias, and arthritis. In addition, pleuritis, pericarditis, and central nervous system involvement are frequently seen in children with SLE. Lupus nephritis develops in the vast majority of affected children and may eventually cause renal failure.

These figures illustrate additional cutaneous features of SLE. The lesions on the back (Fig. 15-3) and the hands (Fig. 15-4) are intensely erythematous macules and slightly edematous papules and plaques. Childhood SLE occurs most frequently during adolescence and is more common among African Americans and among girls. The diagnosis is established when four of eleven clinical and laboratory criteria are met. Clinical criteria include rash (either malar or discoid), photosensitivity, oral ulcers, and disease of the joints, lungs, kidneys, and central nervous system. The single most reliable laboratory test is antinuclear antibody which will be positive in almost every case of childhood SLE. Other laboratory criteria include leukopenia, thrombocytopenia, and the presence of antinative DNA antibodies.

This condition is due to the transplacental passage mainly of anti-Ro (anti-SS-A) antibodies from mother to infant. However, anti-La (anti-SS-B) and anti-RNP antibodies may be implicated. The mother may suffer from a form of connective tissue disease or may be completely asymptomatic. The infants pictured in Figs. 15-5, 15-6, 15-7 were born with these atrophic and telangiectatic plaques on the face. The lesions may also resemble those of subacute cutaneous lupus in the older patient. The skin lesions, which are slightly more common in girls, tend to resolve without scarring. Nonfluorinated topical steroids and avoidance of the sun are the only treatments that may be required.

Figure 15-8 shows atrophic telangiectatic changes that are most often seen on the temple and scalp which may lead to permanent alopecia. A frequent complication of neonatal lupus is congenital heart block that may be found in utero. This situation is potentially life threatening and may sometimes require implantation of a pacemaker. Although neonatal lupus is self-limited, there are reports of ...

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