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Sicca Syndrome: An Unusual Manifestation of Sarcoidosis in Childhood

DANIEL L. COHEN, MC
Am J Dis Child. 1983;137(3):289-290. doi:10.1001/archpedi.1983.02140290075020.
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Sarcoidosis is a chronic, often debilitating, granulomatous, multiple-system disorder that commonly affects adults but only occasionally occurs in childhood. Although it has been previously described as a rare pediatric disease, recent studies have suggested that the incidence of asymptomatic disease may be substantial in certain population groups.1 Pulmonary infiltration and hilar lymphadenopathy are the most frequently encountered early manifestations of sarcoidosis, although cutaneous and ophthalmologic symptoms (uveitis) may also occur in some patients.2,3 The clinical diagnosis is one of exclusion and must be confirmed by a positive Kveim-Siltzbach skin test or by histopathologic evidence of widespread noncaseating epithelioid granulomas in more than one organ.4

The syndrome of keratoconjunctivitis sicca, xerostomia, and salivary gland adenopathy is an unusual isolated manifestation of sarcoidosis and may be confused with Sjögren's syndrome,5 a chronic autoimmune disorder related to rheumatoid arthritis and occasionally associated with malignant lymphoproliferative disease. Histologic differentiation of

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