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Patient Story
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A 4-year-old boy presents with “multiple bumps” that have been growing on his face (Figure 150-1). The differential diagnosis of these lesions included cutaneous sarcoidosis and granuloma annulare. A punch biopsy was performed and the diagnosis of sarcoidosis was made.

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FIGURE 150-1

Papular lesions of sarcoidosis on the face of a 4-year-old boy. (Used with permission from Weinberg SW, Prose NS, Kristal L. Color Atlas of Pediatric Dermatology, 4th edition, Figure 15-23, New York, NY: McGraw-Hill, 2008.)

Graphic Jump Location
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Introduction
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Sarcoidosis is a multisystem granulomatous disease most commonly involving the skin, lungs, lymph nodes, liver, and eyes. There is no clear gender predominance in childhood sarcoidosis.1 Of interest, outside the US sarcoidosis mainly occurs in the predominant race of the country.2 Higher incidences of cases occur in certain parts of the world, including Sweden in Europe and the South Atlantic and Gulf States in the US.35

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In the pediatric population, sarcoidosis is divided into early and late onset. Early onset involves children in their first four years of life, presenting with a triad of arthritis, rash, and uveitis.4 In this patient population, typical pulmonary disease occurs in about 22 percent of children in this age group.6 Early onset sarcoidosis is seen mostly in Caucasian patients and these patients may have a protracted course with severe morbidity and residual impairments.7,8

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Late onset sarcoidosis in children presents as a multisystem disorder, with lung involvement most common.6 Up to 60 percent of children have an abnormal chest x-ray at initial presentation, with the predominant symptom being a mild, dry, chronic cough.6  The eyes are also affected in older children (20 to 30%).3,9 Symptoms include eye redness, blurred vision, photophobia, and ocular pain. Ophthalmic sarcoidosis manifests as uveitis with anterior segment involvement (84% of cases).9 Other complications include optic neuritis, band keratopathy, cataracts, glaucoma, and retinal vasculitis. Other organ systems may be involved including the reticuloendothelial system (enlargement of lymph nodes),1 cutaneous (erythema nodosum),2 musculoskeletal (joint effusions, arthralgias, myositis),2 renal (nephrocalcinosis, abnormal urinalysis),3,10 cardiovascular (arrhythmias, sudden death),11 central nervous system (seizures, cranial neuropathies, diabetes insipidus, growth hormone deficiency),6,12,13 and hepatic (abnormal liver function tests) systems.14

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Synonyms
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  • Juvenile early onset sarcoidosis.

  • Juvenile late onset sarcoidosis.

  • Juvenile systemic granulomatosis.

  • Childhood sarcoidosis.

  • Lupus pernio (sarcoidosis of the face that resembles cutaneous lupus).

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Epidemiology
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  • Rare in pediatric age group; more common in adolescents and young adults.

  • Male/female ratio = 1.

  • Two distinct forms of juvenile sarcoidosis—Early onset and late onset.

    • Early:

      • Presents within the first four years of life.

      • Triad of arthritis/rash/uveitis.

      • Predominantly Caucasian patients.

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