Gottron papule-like skin changes

Description

A 14-year-old girl came to our medical attention for persisting/recurrent fever for several months; that symptom was associated to recurrent diffuse lymphadenopathy and urticarial skin rashes (figure 1). In the previous weeks, she complained of general malaise, diffuse arthralgia and musculoskeletal pain, in addition to persistent low-grade fever. Importantly, she developed also the so-called heliotrope rash (figure 2), being pathognomonic of dermatomyositis, although blood biochemistry showed no significant alterations of creatine kinase (115 U/µL) and aldolase (12 U/L), which represent the most specific serum markers of myopathy. As regards the complete diagnostic work-up, the inflammatory parameters resulted to be increased mildly to moderately and the autoimmunity panel (anti-nuclear antibodies (ANA), extractable nuclear antigens (ENA), anti-double stranded DNA (anti-dsDNA),  cytoplasmic antineutrophil cytoplasmic antibodies (ANCA), perinuclear ANCA, rheumatoid factor (RF), anti-transglutaminases antibody (anti-tTG), cold agglutinins) showed no autoantibody. Moreover, the infectious investigations included:  cytomegalovirus, Epstein-Barr virus (EBV), Toxoplasma gondii, Parvovirus B19, Rickettsia spp, Bartonella...

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