Volume 5, Issue 2, Pages (February 2019)

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Volume 5, Issue 2, Pages 176-179 (February 2019) The pulseless patient: Profound vasculopathy as the presenting feature of fulminant dermatomyositis and response to therapy Farzam Gorouhi, MD, Maija Kiuru, MD, PhD, Marc Silverstein, MD, Parisa Emami-Naeini, MD, MPH, Susanna S. Park, MD, PhD, Danielle Tartar, MD, PhD JAAD Case Reports Volume 5, Issue 2, Pages 176-179 (February 2019) DOI: 10.1016/j.jdcr.2018.11.011 Copyright © 2018 American Academy of Dermatology, Inc. Terms and Conditions

Fig 1 Cutaneous manifestations of JDM. A, Faint violaceous erythema over bilateral eyelids (heliotrope rash). B, Violaceous erythema with fine scale over elbow (Gottron sign). C, Faint flagellate erythema over upper extremities. D, Retiform purpura over bilateral lower extremities. JAAD Case Reports 2019 5, 176-179DOI: (10.1016/j.jdcr.2018.11.011) Copyright © 2018 American Academy of Dermatology, Inc. Terms and Conditions

Fig 2 Histopathologic findings in JDM. A, Punch biopsy of flagellate erythema on left upper arm shows rare necrotic keratinocytes, suggestive of subtle interface dermatitis, compatible with DM. B, Punch biopsy of the right shin shows vascular occlusive disease with fibrin thrombi, compatible with JDM-associated vasculopathy. (Original magnifications: ×40.) JAAD Case Reports 2019 5, 176-179DOI: (10.1016/j.jdcr.2018.11.011) Copyright © 2018 American Academy of Dermatology, Inc. Terms and Conditions

Fig 3 Retinal vasculitis and branch retinal artery occlusion in JDM and response to therapy. Color fundus photographs on initial presentation. A, Right eye. Small, wedge-shaped area of retinal whitening and edema (white arrow) noted inferior to the fovea consistent with branch retinal artery occlusion. B, Left eye with normal findings. Fluorescein angiography on initial presentation. C, Right eye with angiographic leakage of retinal arteries perfusing the inferior macula consistent with retinal vasculitis (red arrow). D, Left eye with mild patches of angiographic leakage from subtle retinal vasculitis despite lack of symptoms. JAAD Case Reports 2019 5, 176-179DOI: (10.1016/j.jdcr.2018.11.011) Copyright © 2018 American Academy of Dermatology, Inc. Terms and Conditions