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Patient Story

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A previously healthy 16-year-old African American girl presented to the clinic with alopecia and rash over her face and arms for 6 months (Figures 173-1 and 173-2). She reported pain in her knees and ankles for a year and an oral ulcer 3 months ago, which had healed. Laboratory evaluation was remarkable for a low white blood cell count at 2.4 × 1000/uL (normal 5.0–13.0 × 100/uL), hemoglobin of 10.8 g/dL (normal 11.8–16.0 g/dL), and platelet count of 109 × 1000/uL (normal 142–424 × 1000/uL). Erythrocyte sedimentation rate was elevated at 45 mm/hr (normal 0–15 mm/hr). Antinuclear antibody was positive at 1:640 with a strongly positive antidouble stranded DNA antibody confirming a diagnosis of systemic lupus erythematosus (SLE). Urinalysis did not show proteinuria to suggest renal involvement. She was treated with prednisone and hydroxychloroquine resulting in improvement in her problems.

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FIGURE 173-1

New onset systemic lupus erythematosus (SLE) presenting with 6 months of a facial rash and alopecia in a 16-year-old African American girl. She also had pain in her knees and oral ulcers. (Used with permission from Vidya Raman, MD.)

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FIGURE 173-2

New onset SLE in a 16-year-old girl (as seen in Figure 173-1) demonstrating her alopecia. Her ANA was 1:640. (Used with permission from Vidya Raman, MD.)

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Introduction

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Systemic lupus erythematosus (SLE) is a chronic inflammatory disease that can affect many organs of the body including the skin, joints, kidneys, lungs, nervous system, and mucus membranes.

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Synonyms

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  • Chronic cutaneous lupus erythematosus = discoid lupus = DLE.

  • Lupus profundus = lupus panniculitis.

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Epidemiology

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  • The true incidence of pediatric SLE is difficult to estimate since there are very few studies in the pediatric population. It is believed that approximately 15 percent of patients with SLE have onset of disease in childhood. Childhood SLE occurs in 6 to 18.9 cases per 100,000 white females, with higher prevalence in African Americans and Hispanics.1

  • The mean age of presentation of SLE in children is 12 to 13 years.

  • Discoid lupus erythematosus (DLE), which develops in up to 25 percent of adults with SLE, is less common in children.3 Patients with only DLE have a 5 to 10 percent risk of eventually developing SLE, which tends to follow a mild course.4 DLE lesions usually slowly expand with active inflammation at the periphery, and then to heal, leaving depressed central scars, atrophy, telangiectasias, and hypopigmentation.5 The female–male ratio of discoid lupus erythematosus (DLE) is 2:1.

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Etiology and Pathophysiology

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  • SLE is a multisystem autoimmune disease with variable presentations. Based on current understanding, an environmental exposure, occurring in a ...

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