Juvenile dermatomyositis: A retrospective review of a 30-year experience

Background: Children with juvenile dermatomyositis (JDMS) have variable ini tial presentations. Objectives Our purpose was to evaluate the epidemiology trends, presenting clinical features, laboratory data, and outcome of patients with JDMS. Methods:A total of 16 patients were identified at Geisinger Medical Center by a 30-year retrospective chart review Results: Sex ratio, age at diagnosis, and outcome were similar to data publ ished in previous studies. However, certain trends were noted. The most com mon initial physical examination findings were an extremity rash (94%) and periungual erythema (75%). New associations of JDMS chat were uncovered inc luded the findings of pruritus (38%) and a psoriasiform scalp dermatitis (2 5%) Nonspecific laboratory elevations were the most common initial laborato ry changes (erythrocyte sedimentation rate, lactate dehydrogenase, and aspa rtate aminotransferase). Tubuloreticular inclusions as found on electron mi croscopy of muscle biopsy specimens were present in all 3 patients tested. One patient with tubuloreticular inclusions had otherwise normal muscle bio psy findings on hematoxylin-and-eosin staining. Two of the 16 patients had cutaneous findings of JDMS but did not exhibit muscle involvement after lon gterm follow-up at 4 and 5 years. Conclusion: Our study confirms that the initial physical and laboratory End ings in patients with JDMS may be nonspecific. The heliotrope rash and Gott ron papules classically associated with dermatomyositis appeared less commo nly than an extremity rash and periungual erythema. Creatinine kinase and a ldolase levels may not be elevated on initial presentation. Pruritus, a pso riasiform scalp dermatitis, End tubuloreticular inclusions Found on muscle biopsy electron microscopy should be additional factors to consider. The lo ng-term follow-up in 2 patients without muscle involvement lends support to the existence of amyopathic dermatomyositis.