A case of overlapping adult‐onset linear scleroderma and Parry‐Romberg syndrome presenting with widespread ipsilateral neurogenic involvement

Abstract

Linear scleroderma is a variant of localized scleroderma. We report a 43-year-old woman who had developed left arm weakness and linear scleroderma on her back during pregnancy at 25 years of age, followed by left hemifacial atrophy and left leg weakness. She had multiple linear scleroderma lesions on her trunk and left limbs, left eyelid ptosis, impairment of vertical movement and abduction of the left eye, left hemifacial atrophy, and weakness and atrophy of the sternocleidomastoid, trapezius, and proximal limb muscles on the left side. On serology, antibodies to U1-ribonucleoprotein and Jo-1 were positive; anti-scleroderma-70 antibody was negative. Skin biopsy demonstrated increased hypertrophic collagen fibers without inflammatory infiltrates. Needle electromyography of left limb muscles revealed mild neurogenic patterns; left quadriceps muscle biopsy showed chronic neurogenic changes. Brain magnetic resonance imaging revealed mild left hemispheric atrophy. This is a rare case of linear scleroderma and Parry-Romberg syndrome presenting with widespread ipsilateral neurogenic manifestations.

Adult-onset en coup de sabre scleroderma in a patient with linear localized scleroderma profunda: A case report and literature review

“…1 To the best of our knowledge, less than 100 cases of patients with AO linear scleroderma ECDS have been reported. 2,4,[5][6][7][8][9][10][11][12][13][14][15][16][17] Patient descriptions of AO localized scleroderma are summarized in Table 1. Of note, while AO ECDS is very rare, ECDS localized scleroderma developing in a patient with pre-existing localized scleroderma is exceedingly rare.…”

“…1 To the best of our knowledge, less than 100 cases of patients with AO linear scleroderma ECDS have been reported. 2,4,5–17…”

Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrier

“…10 LSCS is a very rare condition; most of the scientific literature indicates that it only accounts for 2-4% of linear scleroderma cases in adults, contrasting to the fourth-fold increase in LSCS prevalence in children with 3-17%. 11 Based on an extensive and systematic literature review in Scopus, Web of Science, PubMed and Google Scholar, fewer than 100 case reports have been published on adult-onset LSCS, [12][13][14] with only one case reported in Colombia. 15 In Table 1 and Table 2, we present the case reports, cases series, and systematics reviews of patients with neurological manifestations identified during our literature search.…”