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 pre-existing plaque-morphea, already being treated by balneophototherapy. Further progression of neurological symptoms led to a caesarean section with the delivery of a healthy child...

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 w...

Sir, Linear scleroderma represents a rare disease, more often observed in the pediatric age group. The lower extremities are usually involved; less commonly it affects the frontoparietal area and the anterior scalp. Recently, a large case series of frontoparietal scleroderma en coup de sabre has been reported (1), but in this series, as well as in the English language literature, to the best of...

Acta Neurologica Taiwanica Vol 13 No 4 December 2004 From the Department of Neurology, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, Taiwan. Received November 19, 2004. Revised and Accepted November 29, 2004. Reprint requests and correspondence to: Nai-Shin Chu, MD. Department of Neurology, Chang Gung Memorial Hospital, No. 199, Tung-Hwa N. Road, Taipei, Taiwan. E-mail: chu060@c...

Localized scleroderma is a rare disease, characterized by sclerotic lesions. A variety of presentations have been described, with different clinical characteristics and specific prognosis. In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle, cartilage, and bon...

Received: Jan 09, 2013 Accepted: Jan 16, 2013 Ann Paediatr Rheum 2013; 2:43-49 DOI: 10.5455/apr.011620131348 Introduction Juvenile localized scleroderma ( JLS) is a childhood condition characterized by thickening of the skin. A variety of clinical subtypes have been described, differentiated by the size, shape, and location of skin involvement as well as the presence or absence of extra-cutaneo...

Scleroderma en coup de sabre typically presents with a cutaneous induration, but involvement of the underlying bone, eye, and brain is common. We report on 4 pediatric cases with atypical initial clinical presentations. All cases were seen at the University of California San Francisco. Patients 1 and 2 presented to the Pediatric Rheumatology Clinic with uveitis and orbital pseudotumor in Decemb...

Initially described by Parry in 1825 and systematized by Romberg in 1846, progressive facial hemiatrophy syndrome is a controversial and poorly understood entity, with sporadic occurrence and no known mendelian inheritance. It is a slowly progressive disorder, affecting chiefly females in their first and second decades of life, characterized by unilateral atrophy of facial tissues, mostly skin ...