Linear scleroderma en coup de sabre presenting with positional diplopia and enophthalmos

Abstract

Linear scleroderma en coup de sabre presenting with positional diplopia and enophthalmos A 28-year-old woman with alopecia since childhood presented with 4 years of progressive intermittent diplopia and right eye getting "stuck" with upward gaze. Maddox rod test revealed a subtle intermittent right hypotropia and right enophthalmos (figure 1). Asymptomatic T2 hyperintensities were noted on MRI (figure 2). Scalp morphea was confirmed pathologically. Formal rheumatologic evaluation was otherwise unremarkable. Linear scleroderma en coup de sabre presenting as positional diplopia, unilateral enophthalmos, and focal neuroimaging findings is rare and is suspected to represent a chronic local inflammatory process.1,2 The brain lesions tend to develop adjacent to the skin atrophy. Carry the Only Card that Helps Support the AAN-and Get a $100 Cash Rewards Bonus!Apply for the BankAmericard Cash Rewards TM credit card today to start getting more cash back for the things you buy most-plus a $100 cash rewards bonus offer! Visit AAN.com/view/CashRewards and enter priority code "VACN51." BrainPACBrainPAC is the American Academy of Neurology's (AAN) federal political action committee.• Since its inception, more than 3,300 AAN members have contributed $1,900,000 to BrainPAC.• BrainPAC anticipates contributing $650,000 during the 2015-2016 election cycle placing BrainPAC amongst the ranks of other major medical specialties.• During the 2014 congressional campaign, 89 percent of candidates supported by BrainPAC won their elections.BrainPAC supports both Democrats and Republicans who support issues important to the practice of neurology and the care of patients with neurologic conditions. US AAN members are invited to learn more at BrainPAC.org.

Localized scleroderma causing enophthalmos: A rare entity

“…The ophthalmic manifestations in scleroderma may include ptosis, lid induration or tightening, lash loss, motility disorder, keratoconjunctivitis sicca, conjunctival injection and vascular sludging, fornix shortening, iritis, heterochromia iridis, papillitis, retrobulbar pain, enophthalmos, displacement of outer canthus from resorption of orbital bone, and retinal hemorrhages. [ 2 3 4 5 ]…”

Disorders of Hair

“…• Clinical feature: Linear scleroderma type en coup de sabre typically affects the frontoparietal area of the head. An atrophic furrow of the skin frequently coexists with underlying bone thinning (Lenassi et al 2017, Hock et al 2016Pinho et al 2016) (Figs. 44 and 45).…”