title: A fatal presentation of dermatomyositis with facial swelling.
abstract: Juvenile dermatomyositis (JDM) is the most common inflammatory autoimmune myopathy in children. Most common presentations consist of heliotrophic rash and/or gottron's papules in addition to proximal muscle weakness. A typical presentations have been reported. We present a 13-year-old African American male who presented with a two-week history of bilateral periorbital edema that was unresponsive to glucocorticoids. He had elevated transaminases but no detectable muscle weakness. A muscle biopsy was consistent with juvenile dermatomyositis. This case highlights the need to consider dermatomyositis in cases of facial swelling and the use of aggressive immunosuppressive therapies due to its associated vasculopathies.
authors.affiliation: Departments of Microbiology, Immunology and Biochemistry, University of Tennessee Health Science Center, Memphis, TN 38163, United States.
authors.forename: Nishant
authors.initials: N
authors.lastname: Dwivedi
authors.forename: Christie
authors.initials: C
authors.lastname: Michael
authors.forename: D Betty
authors.initials: DB
authors.lastname: Lew
authors.forename: Sandra
authors.initials: S
authors.lastname: Arnold
authors.forename: Masanori
authors.initials: M
authors.lastname: Igarashi
authors.forename: Tulio
authors.initials: T
authors.lastname: Bertorini
authors.forename: Jerome W
authors.initials: JW
authors.lastname: Thompson
authors.forename: Linda K
authors.initials: LK
authors.lastname: Myers
authors.forename: Monica L
authors.initials: ML
authors.lastname: Brown
mesh_terms: D000293:Adolescent; D001706:Biopsy; D003882:Dermatomyositis / Q000150:complications / Q000175:diagnosis*; D004487:Edema / Q000209:etiology*; D005145:Face; D017809:Fatal Outcome; D006801:Humans; D008297:Male