Morphea in coup sabre

Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. Current treatment paradigms rely on low levels of evidence, primarily case reports and case series-supported by expert opinions. The aim of this article was to systematically analyze current data related to the treatment of localized scleroderma en coup de sabre. The databases Scopus, PubMed, and EBSCO were searched for all reports discussing the treatment of localized scleroderma en coup de sabre...

Children and adolescents with localized scleroderma (LS) are at high risk for extracutaneous-related functional impairment including hemiatrophy, arthropathy, seizures, and vision impairment. Compared with adult-onset LS, pediatric disease has a higher likelihood for poor outcome, with extracutaneous involvement twice as prevalent in linear scleroderma, disease relapses more common, and disease duration more than double. Consensus among pediatric rheumatologists on treating patients at risk for significant morbidity with systemic immunosuppressants has led to major improvements in outcome...

En coup de sabre variant of linear morphea (LM) is a rare sclerotic skin disorder characterized by disfiguring linear depression of the frontal or frontoparietal forehead. Current attempts for cosmetic correction of atrophic lesions must be preceded by an evaluation of disease activity of LM, either by a sufficient clinical assessment or histologic evidence. Corrective procedures including corrective surgery, autologous fat grafting, hyaluronic acid filler injections were performed with varying degrees of success; still, there is a need for treatment options with non-invasive and long-term maintenance effects...

The en coup de sabre variant of linear scleroderma typically occurs in children. We report a unique case of adult-onset en coup de sabre scleroderma in a patient with linear localized scleroderma profunda. The patient was treated with oral steroids and oral methotrexate improving her cutaneous disease. This case highlights the importance of a thorough cutaneous examination as this adult patient developed an entity traditionally believed to occur in childhood.

No abstract text is available yet for this article.

Parry-Romberg syndrome (PRS) is a rare disorder of uncertain etiology that is characterized by progressive atrophy of the soft and hard tissues of face, typically occurring in the first 2 decades of life. It is more commonly seen in females. The disease progresses slowly with gradual atrophy, frequently associated with neurological, ophthalmological, and other system involvement, resulting in secondary complications. The severity of deformity varies depending on the age of onset of disease. Those in whom the disease starts at an earlier age will have more severe deformity...

BACKGROUND: There are still few dermatological studies on morphea. We evaluated the epidemiological and clinical features and management of pediatric morphea, reporting dermatologists experience. METHODS: A multicentre retrospective observational study was carried out on the epidemiological and clinical features and management of the disease between 01/01/2009 and 01/10/2014 in 10 Italian Dermatological Units. RESULTS: We collected the data of 69 children affected by: circumscribed morphea (39...

Scleroderma is a rare connective tissue disorder categorized into systemic sclerosis and localized scleroderma, also called morphea. Linear scleroderma of the scalp, also called en coup de sabre, is infrequently associated with neurologic symptoms. We describe a case of linear scleroderma en coup de sabre in a 28-year-old female presenting with seizures and characteristic cutaneous lesions. Imaging findings over a course of 7 years demonstrated waxing and waning signal changes. MR perfusion and spectroscopic imaging, demonstrating decreased cerebral blood volume, increased mean transit time, and decreased metabolites, was performed during a time of progressing radiological and clinical findings...

En coup de sabre morphea is a clinical variant of morphea, presenting as a linear depressed, atrophic area in the paramedian forehead or the frontoparietal scalp, resembling a stroke from a sword. It affects the skin and subcutaneous connective tissues, with possible extension to the underlying musculature, cartilage, and bone and variable association with neurologic symptoms. A 50-year-old woman presented to our clinic for evaluation of an atrophic lesion on her forehead and scalp appearing 1 year before, progressively extending over time...

Linear scleroderma en coup de sabre (LSCS) is a clinical variant of morphea that presents with a linear alopecic patch over the frontal scalp. Linear alopecia areata may present as a close differential in the incipient stages of LSCS in the pediatric population, especially when the binding down and pigmentary changes characteristic of LSCS have not developed. Histopathology may also be noncontributory in such scenarios. We hereby report a case of linear alopecia in a 5-year-old Indian girl and highlight the role of trichoscopic evaluation in clinching the diagnosis...

BACKGROUND: Linear cutaneous lupus erythematosus (LE) is an unusual form of LE-specific cutaneous condition, occurring in children and young adults. Due to its rarity, the diagnosis of linear cutaneous LE can be difficult and facial lesions can resemble linear morphea or en coup de sabre. Differential diagnosis of similar conditions along the lines of Blaschko must be differentiated from linear LE. CASE PRESENTATION: We report a case of linear discoid LE on the forehead of an adult female Thai patient mimicking en coup de sabre...

BACKGROUND: Parry-Romberg syndrome, also known as progressive hemifacial atrophy, is a rare degenerative disorder with numerous distinctive clinical presentations. It is usually slowly progressive, occurring more in females, and affects primarily one side of the face; it causes unilateral atrophy and loss of skin, subcutaneous tissue, muscles, and bones, and can even extend to oral structures. Other involvements that can occur are ocular and neurological; however, it is frequently associated with linear scleroderma, known as en coup de sabre...

BACKGROUND: Congenital morphea is a form of localized scleroderma that presents at birth. There is limited information on its presentation and progression. METHODS: Patients with congenital morphea were identified from five pediatric dermatology and rheumatology tertiary care centers in Canada, the United States, and Italy from 2001 to 2016. Cases from the literature were identified by searching Ovid (EMBASE and MEDLINE) from inception to June 30, 2017. Disease characteristics and prevalence of extracutaneous involvement were analyzed...

Linear scleroderma "en coup de sabre" is a subset of localized scleroderma with band-like sclerotic lesions typically involving the frontoparietal regions of the scalp. En coup de sabre and Parry-Romberg syndrome are variants of linear morphea on the head and neck that can be associated with neurologic manifestations. On imaging, patients may have lesions in the cerebrum ipsilateral to the scalp abnormality. We present a case of an 8-year-old girl with a left frontoparietal "en coup de sabre" scalp lesion and describe the neuroimaging findings of frontoparietal white matter lesion discovered incidentally on routine magnetic resonance imaging...

Linear scleroderma en coup de sabre (LSCS) is the most common form of morphea on the scalp and its trichoscopic features have not been described yet. We report 2 adult women with LSCS with distinct dermoscopic findings. They both had an atrophic well-defined linear patch of alopecia on the frontotemporal scalp. Dermoscopy showed loss of follicular openings on a whitish skin surface; scattered black dots, broken hairs, and pili torti; and short thick linear and branching tortuous vessels on the periphery of the lesion...

Parry-Romberg syndrome (PRS) may overlap localized scleroderma (morphea) lesions with linear depression (en coup de sabre [ECDS]). Overlap case with PRS and ECDS was presented. Enophthalmos, uveitis, ocular torticollis, keratic linear precipitates, and anti-double-stranded DNA positivity were identified. Subendothelial keratic precipitates detected by an in vivo laser scanning confocal microscopy were the first profiled in the literature. Patients must be evaluated and followed up carefully by their clinics to prevent misdiagnosis and unnecessary procedures such as surgery of ocular torticollis as muscular torticollis...

Scalp morphea presents as a scarring alopecia in en coup du sabre pattern. We report an unusual presentation of a round hairless patch of morphea on the occipital scalp present for 15 years. The scalp lesion aligned with 2 other hyperpigmented lesions of biopsy-proven morphea in the lower back. Pathology of horizontal sections from the scalp lesion showed follicular dropout, thickening of the collagen bundles, and preserved eccrine and follicular structures. Marked lymphocytic perineural infiltrate, a reported clue to the diagnosis of scalp morphea, contributed to the diagnosis...

BACKGROUND: En coup de sabre is a rare subtype of linear morphea, located on the forehead or frontoparietal scalp. Systemic treatment of localised morphea with methotrexate has been reported in a few clinical reports. However, there are no case series using methotrexate treatment for En coup de sabre. OBJECTIVE: To evaluate the efficacy and tolerability of methotrexate in the treatment of en coup de sabre linear morphea subtype. METHOD: A retrospective chart review was performed for paediatric and the adult patients with en coup de sabre evaluated in the Dermatology Clinic at Wake Forest University School of Medicine treated with methotrexate...

We describe a case of Parry Romberg syndrome/ en coupe de sabre in a woman whose disease started as seizures at age 8 but was diagnosed at the age 39. During these 31 years she got married, completed a first degree at university, had two successful pregnancies and has been gainfully employed. The features of generalized tonic-clonic seizures, autoimmune abnormalities, ocular abnormalities, morphea en coup de sabre and brain imaging abnormalities were present. Areas of parietal lobe cerebral calcification were encountered on the computed tomographic scan and bilateral periventricular white matter changes on the magnetic resonance imaging with frontal, temporal and parietal lobe brain atrophy ipsilateral to the facial hemiatrophy...

We report the case of a young girl who presented with hemiparesis, seizures, and subtle features consistent with a linear form of facial morphea (en coup de sabre). She was treated with pulsed parenteral steroids and oral steroids and started on methotrexate. Magnetic resonance imaging results and neurologic problems improved after 6 months. Switching off inflammation early in the course of disease seemed to reverse some of the central nervous system changes. Assessment of children with unexplained hemiparesis and seizures should include careful examination of the face and scalp, looking for subtle signs of skin change and asymmetry...