Juvenile dermatomyositis with Sjögren's syndrome.
Juvenile dermatomyositis (JDM) is a rare disease, and Sjögren's syndrome (SS) is unusual in adolescents. We report the first case of biopsy-proven JDM and SS with pulmonary involvement. A 15-year-old adolescent boy presented with recurrent parotid gland hypertrophy, severe muscle weakness, pronounced skin rash and widespread lymphadenopathy. JDM was diagnosed by clinical examination, elevated muscle enzymes, electromyography and muscle biopsy; SS was diagnosed by xerostomia, anti-Ro (SS-A) positivity and histopathological analysis of salivary gland tissue. This case illustrates a systematic approach which we feel is especially important in the younger patient with a more plastic immune system.
Holmes
M V
MV
Department of Adolescent Rheumatology, Centre for Rheumatology, University College London Hospital, 3rd Floor Central, 250 Euston Road, London NW1 2PQ, UK.
Ioannou
Y
Y
Borysiewicz
C
C
Sen
D
D
D000293:Adolescent; D003882:Dermatomyositis / Q000150:complications* / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000209:etiology / Q000473:pathology; D008297:Male; D010306:Parotid Gland / Q000473:pathology*; D011658:Pulmonary Fibrosis / Q000209:etiology / Q000473:pathology; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis / Q000473:pathology*