Linear scleroderma "en coup de sabre" coexisting with plaque-morphea: neuroradiological manifestation and response to corticosteroids

Abstract

A 24 year old woman in the 33rd week of pregnancy developed progressive neurological complications with right sided hemiparesis in association with the occurrence of linear scleroderma "en coup de sabre" (LSCS) and preexisting plaque-morphea, already being treated by balneophototherapy. Further progression of neurological symptoms led to a caesarean section with the delivery of a healthy child. Brain magnetic resonance imaging (MRI) showed focal T2 signal increases in the left frontoparietal region directly adjacent to the area of LSCS. Cerebrospinal fluid analysis revealed oligoclonal bands, suggesting an intracerebral inflammatory process. Subsequent pulsed corticosteroid treatment led to a remission of neurological symptoms and to a marked resolution of the MRI lesions.

Concomitant en coup de sabre and plaque type morphea in the same patient: a rare occurrence

“…Unterberger et al described a 24-year-old woman in the 33 rd week of pregnancy who developed right-sided hemiparesis and progressive neurological complications in association with linear scleroderma en coup de sabre. She also had pre-existing plaque morphea for which she was receiving balneotherapy [2]. In contrast, our patient had no neurological manifestations and plaque morphea postdated en coup de sabre.…”

“…opisali przypadek 24-letniej kobiety w 33. tygodniu ciąży, u której wystąpił niedowład połowiczy prawostronny i postępujące zaburzenia neurologiczne powiązane z twardziną linijną en coup de sabre. U pacjentki wcześniej stwierdzono twardzinę plackowatą, leczoną balneoterapią [2]. U przedstawionej pacjentki nie odnotowano żadnych objawów neurologicznych, a twardzinę plackowatą zaobserwowano dopiero po rozpoznaniu twardziny en coup de sabre.…”

“…Twardzina linijna może być umiejscowiona na czole i okolicy czołowo--ciemieniowej owłosionej skóry głowy, w której to lokalizacji zwana jest twardziną linijną en coup de sabre, ponieważ zmiany na skórze przypominają wyglądem ślad po cięciu szablą [1]. Do tej pory opisano jedynie kilka przypadków współistnienia twardziny en coup de sabre i twardziny plackowatej [2].…”

“…Linear scleroderma may involve the forehead and fronto-parietal area of the scalp, where it is called linear scleroderma en coup de sabre because the appearance of skin lesions simulates the stroke of a saber [1]. Co-existence of en coup de sabre and plaque morphea has been described in very few case reports in the literature [2].…”

Linear Scleroderma en coup de sabre With Associated Neurologic Abnormalities

“…2 Unlike most forms of localized scleroderma, which lack extracutaneous manifestations, a subset of linear scleroderma referred to as en coup de sabre has been associated with several neurologic abnormalities. [3][4][5][6][7][8][9] Many of the epidemiologic studies of scleroderma have come from dermatology and rheumatology centers, where referral bias influences estimates of the incidence, prevalence, and severity of this disease. Systemic scleroderma is rare in the pediatric population, and localized scleroderma has been estimated to be at least 10 times more common than systemic disease.…”