Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease?

Хамаганова, И. В.

doi:10.3389/fmed.2017.00258

Cited by 15 publications

(18 citation statements)

References 114 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Interestingly, cranial muscles innervated by ipsilateral cranial nerves III, VI, VII, and XI were also affected in our patient. Involvement of cranial nerves II, III, and VII has been reported in a few cases of scleroderma en coup de sabre . In addition, our patient had left hemispheric brain atrophy, which was reported to occur in 17% of PRS patients .…”

Section: Discussionsupporting

confidence: 54%

“…However, scleroderma en coup de sabre causes hemifacial atrophy predominantly involving the scalp and forehead, whereas our patient had involvement of the entire left half of her face without facial skin lesions, findings suggesting concurrent PRS. Indeed, such coexistence of linear scleroderma and PRS has repeatedly been reported in the literature …”

Section: Discussionsupporting

confidence: 52%

“…Parry–Romberg syndrome (PRS), also known as progressive hemifacial atrophy, is a rare disorder that causes unilateral facial atrophy. Because these conditions present with overlapping manifestations and preferentially affect females in the first 20 years of life, similar pathological mechanisms are assumed . Linear scleroderma occasionally accompanies myositis or myopathy, whereas about one‐fifth of PRS patients have unilateral limb atrophy, mostly ipsilateral to their facial atrophy .…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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

et al. 2019

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionsupporting

confidence: 54%

Section: Discussionsupporting

confidence: 52%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

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

et al. 2019

View full text Add to dashboard Cite

show abstract

“…Twardzina typu cięcia szablą (en coup de sabre -ECDS) objawia się obecnością linijnego zaniku i/lub stwardnienia skóry, tkanki podskórnej, mięśni, kości i przebiega zwykle od poziomu górnej krawędzi brwi do owłosionej skóry głowy, gdzie pojawia się ognisko łysienia bliznowaciejącego [20,21]. Znane są opisy pacjentów z ECDS, u których stwierdzono szerzenie się wykwitów poniżej linii brwi, z zajęciem powiek, rzęs lub skóry nosa [22].…”

Section: Twardzina Typu Cięcia Szabląunclassified

“…En coup de sabre (ECDS) manifests itself by the presence of linear atrophy and/or hardening of the skin, subcutis, muscles, and bones; it usually starts at the level of the upper eyebrow ridge and reaches the scalp, where a cicatricial alopecia focus appears [20,21]. There are known descriptions of patients with ECDS, in whom eruptions spread below the eyebrows involving the eyelids, eyelashes or the skin on the nose [22].…”

Section: En Coup De Sabrementioning

confidence: 99%

Localized scleroderma (morphea). Diagnostic and therapeutic recommendations of the Polish Dermatological Society

Krasowska¹,

Rudnicka²,

Dańczak‐Pazdrowska³

et al. 2019

View full text Add to dashboard Cite

Localized scleroderma (morphea) is a chronic autoimmune disease of the connective tissue. Its etiopathogenesis is unknown. Most often the disease affects the skin, but may also involve the subcutis, muscles, and the osteoarticular system. The clinical presentation and the course of disease may be diverse. This article presents clinical characteristics of various types of the disease, the classification of its subtypes, recommendations for differential diagnosis and treatment. STReSzCzenie Twardzina ograniczona (morphea) jest przewlekłą chorobą zapalną tkanki łącznej o podłożu autoimmunologicznym i o niewyjaśnionej etiopatogenezie. Najczęściej zajęta jest skóra, ale proces chorobowy może zajmować tkankę podskórną, mięśnie i układ kostno-stawowy. Zróżnicowany obraz kliniczny choroby wymaga prawidłowego rozpoznania i zastosowania odpowiedniej terapii. W artykule przedstawiono charakterystykę kliniczną oraz zalecenia dotyczące klasyfikacji, postępowania diagnostycznego i leczenia.

show abstract

Clinical and therapeutic course in head variants of linear morphea in adults: a retrospective review

et al. 2022

View full text Add to dashboard Cite

Parry Romberg Syndrome (PRS) and en coup de sabre (ECDS) are head variants of linear morphea with functional and structural implications. This study describes the clinical course, autoimmune co-morbidities, complications, and treatment of adults with PRS/ECDS at a tertiary referral center. We retrospectively reviewed the records of all 34 adult patients with PRS/ECDS identified through billing code search and seen by dermatologists at our institution between 2015 and 2021. Eight patients (23.5%) had ECDS, 8 (23.5%) had PRS, and 18 (52.9%) had overlap. Twenty-six patients (76.5%) reported ocular, oral, and/or neurologic symptoms, and 8 (23.5%) had concomitant autoimmune/inflammatory conditions. Sixteen patients (47.1%) had a skin biopsy, and 25 (73.5%) had imaging. Forty-six MRIs were obtained, of which 6 (13.0%) reported intracranial findings and 25 (54.3%) reported disease-related connective tissue damage. Twenty-four patients (70.6%) underwent systemic treatment during their disease course per available clinical records. Seventeen patients (70.8%) had improved or stable disease upon treatment completion, with an average duration of 22.2 months. Ten patients (41.7%) reported recurrence of disease following the treatment course. To address changes to facial contour, 6 patients (17.6%) opted for procedural treatments. One patient (16.7%) experienced morphea reactivation following a filler injection performed off-immunosuppression. Compared to findings in children, our study suggests adults with PRS/ECDS are more likely to have oral and ocular complications but experience less severe neurologic symptoms. While systemic treatments appear beneficial in most adult patients with PRS/ECDS, disease may recur following discontinuation.

show abstract

Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease?

Cited by 15 publications

References 114 publications

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

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

Localized scleroderma (morphea). Diagnostic and therapeutic recommendations of the Polish Dermatological Society

Clinical and therapeutic course in head variants of linear morphea in adults: a retrospective review

Contact Info

Product

Resources

About