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Esclodermia cutanea

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By Eduardo Roque Cardiologista com foco em cardiologia hospitalar e cuidados intensivos. Professor de clínica médica.
Ligia Stănescu, Alina Vâlcea, Carmen Florina Popescu, Carmen Elena Niculescu, O Ciobanu, G Călin
We present the case of a girl, aged 8-year-old, with a history of acrocyanosis and repeated respiratory infections with beta-hemolytic streptococcus, which was consulted for the presence of skin lesions in the right buttock area. Clinical examination showed, in the right buttock region, an oval plaque with a diameter about 12 cm, hard, well defined, with irregular outline. The biopsy was performed and it revealed typical aspects of plaque-type morphea. The epidermis was mostly atrophic, with areas of ridge reduction; an important proliferation of collagen fibers within superficial and deep dermis and an abundant lymphocytic inflammatory infiltrate throughout the dermal thickness reaching hypodermic level and infiltrating it...
2010: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
Aida Khaled, Monia Kharfi, Anissa Zaouek, Soumaya Rameh, Rachida Zermani, Becima Fazaa, Mohamed Ridha Kamoun
We report a new case of postvaccination morphea profunda (MP) in a child and discuss its different clinical presentations, prognosis, and therapy and its relationship with "solitary morphea profunda." A 2-year-old healthy girl presented with an induration of the anterior aspect of the left thigh of 9 months duration. The lesion had appeared 3 months after a third dose of diphtheria-tetanus-pertussis vaccine. Cutaneous examination showed an induration of 7 × 7 cm with an "orange peel" texture after pinching the skin...
July 2012: Pediatric Dermatology
Daniel Condie, Daniel Grabell, Heidi Jacobe
OBJECTIVE: Few studies have examined outcomes in adults with pediatric-onset morphea. The objective of the present study was to compare clinical outcomes and health-related quality of life (HRQOL) in adults with onset of morphea in childhood to those in patients with adult onset of morphea. METHODS: Participants in the study were drawn from the Morphea in Adults and Children cohort and included 68 adults with pediatric-onset morphea and 234 patients with adult-onset morphea...
December 2014: Arthritis & Rheumatology
I Pérez-López, C Garrido-Colmenero, R Ruiz-Villaverde, J Tercedor-Sánchez
No abstract text is available yet for this article.
May 2015: Actas Dermo-sifiliográficas
John C Browning
This article outlines the epidemiology, pathogenesis, clinical presentation, diagnosis, and treatment of childhood morphea. Also known as localized scleroderma, morphea is a fibrosing disorder of the skin and subcutaneous tissues. Morphea is differentiated from systemic sclerosis (scleroderma) based on the absence of sclerodactyly, Raynaud phenomenon, and nail-fold capillary changes. Confusion may occur because patients with morphea often have systemic symptoms such as malaise, fatigue, arthralgias, myalgias, and positive autoantibodies...
April 2013: Dermatologic Clinics
Stéphanie Christen-Zaech, Miriam D Hakim, F Sule Afsar, Amy S Paller
BACKGROUND: Morphea is an autoimmune inflammatory sclerosing disorder that may cause permanent functional disability and disfigurement. OBJECTIVES: We sought to determine the clinical features of morphea in a large pediatric cohort. METHODS: We conducted a retrospective chart review of 136 pediatric patients with morphea from one center, 1989 to 2006. RESULTS: Most children showed linear morphea, with a disproportionately high number of Caucasian and female patients...
September 2008: Journal of the American Academy of Dermatology
Nicole Fett, Victoria P Werth
Morphea is a rare fibrosing disorder of the skin and underlying tissues. The underlying pathogenesis of morphea is not completely understood at this time, but ultimately results in an imbalance of collagen production and destruction. Evidence-based treatment options of morphea are limited secondary to the rarity of the disease, and the lack of universally used validated outcome measures. The most commonly used outcome measures are skin scores, computerized surface area measurement, durometer, cutometer, thermography, and ultrasound measurements...
February 2011: Journal of the American Academy of Dermatology
Nicole Fett, Victoria P Werth
Morphea, also known as localized scleroderma, is a rare fibrosing disorder of the skin and underlying tissues. Morphea is differentiated from systemic sclerosis based on the absence of sclerodactyly, Raynaud phenomenon, and nailfold capillary changes. Patients with morphea commonly have systemic symptoms, such as malaise, fatigue, arthralgias, and myalgias, as well as positive autoantibody serologies. However, involvement of morphea is almost uniformly limited to those tissues derived from the mesoderm. The underlying pathogenesis of morphea is incompletely understood at this time, but ultimately results in an imbalance of collagen production and destruction...
February 2011: Journal of the American Academy of Dermatology
Sarah Nouri, Heidi Jacobe
There is huge variation in the evaluation, diagnosis, and treatment of patients with morphea (localized scleroderma). In part, this variability results from the lack of validated methods to assess severity and outcomes with a consequent lack of adequate therapeutic trials. Evaluation is also hindered by lack of information regarding the impact of morphea on patients. Recent studies are addressing this gap in knowledge and include: development of clinical outcome measures, validation of imaging studies, publication of consensus treatment plans, and increased understanding of the impact of morphea on patients and parents...
February 2013: Current Rheumatology Reports
Lisa Weibel, John I Harper, Kevin J Howell
No abstract text is available yet for this article.
May 2007: Journal of Pediatrics
César Magro-Checa, Jacinto Orgaz-Molina, Sunna R Lafèbre, José L Rosales-Alexander, Salvador Arias-Santiago, Enrique Raya
No abstract text is available yet for this article.
May 2015: Cleveland Clinic Journal of Medicine
Julio C Sartori-Valinotti, Megha M Tollefson, Ann M Reed
Morphea and systemic sclerosis are fibrosing disorders of the skin that share common inflammatory and immunologic pathways that are responsible for the vascular changes, increased collagen production, and extracellular matrix proliferation seen in both conditions. Recent advances in molecular biology techniques have furthered our knowledge of the potential underlying pathogenic mechanisms and offer new and provocative areas of research for novel diagnostic and therapeutic interventions. This review focuses on the role of vascular injury in the development of morphea, the use of ultrasonography as a diagnostic modality, and well-established and newly proposed treatments...
2013: Autoimmune Diseases
Nicole M Fett
Morphea is a rare fibrosing disorder of the skin. Evidence-based treatment strategies in morphea are lacking. This review summarizes the available data on morphea treatment and provides therapeutic strategies based on morphea subtypes. The Cochrane Library, Medline and Embase from inception until May of 2011 were searched using the key words "morphea" and "morphea treatment." Reference lists of the resultant articles, as well as relevant reviews, were also searched. This review focuses on randomized controlled trials, prospective interventional trials without controls and retrospective reviews with greater than five subjects...
March 2012: Indian Journal of Dermatology, Venereology and Leprology
Elena Pope, Ronald M Laxer
Morphea (localized scleroderma) is a rare fibrosing disorder of the skin and underlying tissues characterized by skin thickening and hardening due to increased collagen deposition. The significance of the disease depends on the extent of the lesions, potential for functional disability or cosmetic disfigurement, and presence or absence of extracutaneous manifestations. Treatment is tailored; circumscribed forms may require only topical treatment, while forms causing functional impairment or severe cosmetic change may require systemic treatment...
April 2014: Pediatric Clinics of North America
Brittany A Zwischenberger, Heidi T Jacobe
BACKGROUND: Morphea (localized scleroderma) is a skin disorder with significant morbidity. No consistent recommendations exist for therapy, impeding patient care. OBJECTIVE: We sought to create an evidence-based therapeutic algorithm. METHODS: We reviewed English-language literature using search engines and hand searches for therapeutic interventions in morphea. Results were summarized. RESULTS: Narrowband ultraviolet B is appropriate for progressive or widespread superficial dermal lesions; broadband ultraviolet A/ultraviolet A-1 is appropriate for widespread or progressive deeper dermal lesions...
November 2011: Journal of the American Academy of Dermatology
Andrew Kim, Nicholas Marinkovich, Rebecca Vasquez, Heidi T Jacobe
OBJECTIVE: Pansclerotic morphea is a poorly described form of morphea with little information on prevalence, demographics, and clinical features. Classification criteria for this subtype varies and the distinction from other forms of morphea, such as extensive generalized morphea and pansclerotic morphea, is not always clear. The purpose of our study was to clarify classification criteria for pansclerotic morphea by identifying its prevalence in the morphea in adults and children (MAC) cohort and describing its demographic and clinical features as compared with generalized morphea...
January 2014: Journal of Rheumatology
Kathryn S Torok
Pediatric scleroderma includes 2 major groups of clinical entities, systemic sclerosis (SSc) and localized scleroderma (LS). Although both share a common pathophysiology, their clinical manifestations differ. LS is typically confined to the skin and underlying subcutis, with up to a quarter of patients showing extracutaneous disease manifestations such as arthritis and uveitis. Vascular, cutaneous, gastrointestinal, pulmonary, and musculoskeletal involvement are most commonly seen in children with SSc. Treatment of both forms targets the active inflammatory stage and halts disease progression; however, progress needs to be made toward the development of more effective antifibrotic therapy to help reverse disease damage...
April 2012: Pediatric Clinics of North America
Nicole M Fett
No abstract text is available yet for this article.
September 2013: JAMA Dermatology
Carolina Tomiyoshi, Adma Silva de Lima Wojcik, Elisa Milani Oba Vencato, Guilherme Ribas Taques, José Fillus Neto, Fabiane Andrade Mulinari Brenner
Localized scleroderma or morphea is a chronic disease of the connective tissue. Its etiology may be autoimmune and the condition results from a disturbance in collagen synthesis and deposition, clinically represented by sclerotic skin lesions. Some plaques may be yellowish, which can be misleading at diagnosis. This article reports the case of an adolescent girl who concomitantly presented erythematous lesions and yellowish lesions, both of which constitute clinical manifestations of the disease.
May 2010: Anais Brasileiros de Dermatologia
I Bielsa Marsol
Morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. It comprises a number of subtypes differentiated according to their clinical presentation and the structure of the skin and underlying tissues involved in the fibrotic process. However, classification is difficult because the boundaries between the different types of morphea are blurred and different entities frequently overlap. The main subtypes are plaque morphea, linear scleroderma, generalized morphea, and pansclerotic morphea...
October 2013: Actas Dermo-sifiliográficas
2015-09-12 04:06:17
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