P. Treadwell et al. (eds.)Atlas of Adolescent Dermatologyhttps://doi.org/10.1007/978-3-030-58634-8_15

15. Systemic Lupus Erythematosus

Patricia Treadwell¹

(1)

Department of Dermatology, Indiana University School of Medicine, Indianapolis, IN, USA

Patricia Treadwell

Email: ptreadwe@iupui.edu

Keywords

Systemic lupus erythematosus (SLE)Discoid lupus lesionsHydroxychloroquine

15.1 Introduction

Systemic lupus erythematosus (SLE) is a chronic inflammatory condition that can affect various organ systems. The version of this disorder, which occurs in childhood, is labeled as childhood-onset systemic lupus erythematous (cSLE).

15.2 Epidemiology

Childhood-onset SLE (cSLE) has its peak incidence in early adolescence. The average age of presentation of cSLE is 12–14 years. M:F ratio is 1:5 prior to puberty. Following puberty, the ratio is 1:9. Although no registry exists, estimates of the incidence are typically quoted as 3.3–8.8 per 100,000 individuals. It has been noted that cSLE affects children of Black, Hispanic, and Asian ancestry more often, and they have a worse prognosis.

15.3 Clinical Findings

The most common cutaneous findings are photosensitivity, malar erythema (Fig. 15.1), discoid LE lesions (Fig. 15.2) , nail fold telangiectasias (Fig. 15.3), and oral ulcers. Subacute cutaneous lupus is characterized by scaly erythematous lesions in sun-exposed area such as the back (Fig. 15.4). Other findings include alopecia, livedo reticularis, Raynaud phenomenon, and panniculitis. Extracutaneous features include fever, arthralgias, arthritis, fatigue, and CNS involvement.

../images/446896_1_En_15_Chapter/446896_1_En_15_Fig1_HTML.jpg — Fig. 15.1
SLE 3 Erythema of the malar areas and nose

../images/446896_1_En_15_Chapter/446896_1_En_15_Fig2_HTML.png — Fig. 15.2
DLE 1 Somewhat annular lesion with scale, hypopigmentation, and atrophy

../images/446896_1_En_15_Chapter/446896_1_En_15_Fig3_HTML.jpg — Fig. 15.3
Cuticles show dilated capillaries

../images/446896_1_En_15_Chapter/446896_1_En_15_Fig4_HTML.jpg — Fig. 15.4
(a) Scle Erythematous scaly patches of the back. (b) Scle2 Scaly patch in close up

15.4 Laboratory

In 99% of children with cSLE, the antinuclear antibody (ANA) is positive. If the titer is greater than 1:1280 or higher, cSLE is a likely diagnosis.

The anti-dsDNA antibodies can be used to monitor disease activity. The anti-dsDNA and cardiolipin antibodies are more frequent in cSLE as compared to aSLE (adult-onset SLE); however, a positive rheumatoid factor is less common.

Urinalysis is an important tool to evaluate any renal involvement that may develop.

15.5 Treatment

Treatment options for cSLE include oral hydroxychloroquine, glucocorticosteroids, and other immunosuppressants, including biologics. The cutaneous lesions may be treated with topical or intralesional steroids or with topical calcineurin inhibitors.

15.6 Prognosis

Adolescents with cSLE have a good prognosis when there is no renal involvement. Those adolescents with renal involvement require more aggressive therapies and close follow-up.