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1.
Autoimmun Rev ; 20(2): 102730, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33338593

RESUMO

Systemic sclerosis (SSc) is considered one of the most challenging and difficult to treat among rheumatic disorders, due to its severity, multiorgan manifestation and different outcomes. It manifests fibrosis in different organs, mostly in skin and lungs. The skin fibrosis expression is considered the first sign of the disease and usually it is followed by internal organ fibrosis. An aberrant immune system activation seems to relate to the expression of the disease, but even environmental influences and dysregulation of many molecules signalling pathways are involved in the development of the disease. Current therapies are limited and characterized by multiple side effects: systemic route is the elective administration route, which decreases patient adherence to the therapy, as they are often already bothered by pain and disfigurement. Treatments available are organ-based, originally indicated for other conditions and there is no therapy available to reduce the fibroblast population size within existing fibrotic lesions. Disease-modifying therapies or immunomodulatory agents that are highly effective in other rheumatic diseases have shown disappointing results in SSc. There are thus no standardized and effective treatments for this disease, and there are even unanswered questions related to the insurgence of the pathology and all the mechanisms involved. An ideal approach could be considered "targeted therapy" that will be an increasingly attainable objective insofar as our understanding of the disease improves. The advantages in identifying the molecule and the signalling pathways involved in the pathology have helped to find some novel compounds for the therapy of scleroderma fibrosis or following innovative uses for already-approved drugs, corroborated by many clinical studies.


Assuntos
Esclerodermia Localizada , Escleroderma Sistêmico , Fibroblastos , Fibrose , Humanos , Esclerodermia Localizada/patologia , Escleroderma Sistêmico/tratamento farmacológico , Escleroderma Sistêmico/patologia , Pele/patologia
3.
Medicine (Baltimore) ; 99(41): e22582, 2020 Oct 09.
Artigo em Inglês | MEDLINE | ID: mdl-33031308

RESUMO

RATIONALE: Systemic sclerosis (SSc) is a serious multisystem connective tissue disease. When SSc is accompanied by systemic lupus erythematosus (SLE), called SSc-SLE overlap syndrome. SSc associated thrombotic microangiopathy (SSc-TMA) can lead to scleroderma renal crisis, it mainly manifests hypertension or even malignant hypertension, acute kidney injury, and higher mortality. The case of SSc-SLE overlap syndrome combined with SSc-TMA has rarely been reported. PATIENT CONCERNS: We report the case of an elderly male with SSc-SLE overlap syndrome combined with scleroderma renal crisis and SSc-TMA. DIAGNOSES: The patient has typical of SSc on the face and hands, combined with pulmonary artery hypertension, interstitial lung disease, heart failure and malignant hypertension, as well as SLE, lupus nephritis class V, and TMA, which were definitively diagnosed by clinical laboratory examination and renal histopathology. INTERVENTIONS: The patient was treated with prednisone, cyclophosphamid, renin-angiotensin system inhibitors, diuretics, and acetylcysteine. OUTCOMES: The patient died suddenly of heart failure on the 35th day after discharge. LESSONS: The occurrence of TMA leads to the deterioration of the prognosis of SSC-SLE overlap syndrome. The diagnosis of SSC-TMA in SSc-SLE overlap syndrome depends on clinical laboratory examination and renal histopathology.


Assuntos
Lúpus Eritematoso Sistêmico/complicações , Esclerodermia Localizada/complicações , Escleroderma Sistêmico/complicações , Microangiopatias Trombóticas/complicações , Humanos , Masculino , Pessoa de Meia-Idade
4.
Reumatol. clín. (Barc.) ; 16(5,pt.1): 356-358, sept.-oct. 2020. ilus, tab
Artigo em Inglês | IBECS | ID: ibc-195893

RESUMO

Case history of a small girl outlet with epilepsy, followed by scleroderma skin damage and uveitis, neurovasculitis with white matter foci in brain on the side of skin lesion in two months, immunologic disease activity. Resistance to conventional immunosuppressive therapy forced us to initiate the treatment with tocilizumab. It was well tolerated and led to significant improvement of brain, ocular and skin manifestations


Historia de la enfermedad de una niña con epilepsia, así como las lesiones cutáneas de la esclerodermia y la uveítis, la neurovasculitis con materia blanca se centra en el cerebro en el lado de la lesión de la piel en 2 meses, la actividad de las enfermedades inmunológicas. La resistencia a la terapia inmunosupresora tradicional nos hizo comenzar el tratamiento con tocilizumab. Fue bien tolerado y condujo a una mejoría significativa en las manifestaciones cerebrales, oculares y de la piel


Assuntos
Humanos , Feminino , Pré-Escolar , Esclerodermia Localizada/complicações , Esclerodermia Localizada/tratamento farmacológico , Epilepsia/complicações , Uveíte/complicações , Anticorpos Monoclonais Humanizados/uso terapêutico , Esclerodermia Localizada/diagnóstico , Eletroencefalografia , Doenças Autoimunes do Sistema Nervoso , Imagem por Ressonância Magnética
5.
Clin Exp Rheumatol ; 38 Suppl 125(3): 140-147, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32865172

RESUMO

OBJECTIVES: To evaluate the diagnostic value of hand ultrasound (US) in systemic sclerosis (SSc) and to explore its relevance within a combined diagnostic approach. METHODS: 224 patients with suspected SSc were consecutively included. They all had US evaluation assessing the presence of fibrotic tenosynovitis (fibrotic TS) and ulnar artery occlusion (UAO). The final diagnosis of SSc was based on the clinical evaluation of a board of experts independently of any pre-established classification criteria. RESULTS: 166 patients were finally diagnosed as SSc according to the experts as reference standard. 62 SSc and 8 non-SSc patients had UAO (uni or bilateral) (p=0.001). 23 SSc patients and 1 non-SSc patient had US fibrotic TS (p=0.007). A US SSc-pattern (presence of UAO and/or fibrotic TS) was reported in 73 SSc patients and 9 non-SSc patients (p<0.001). UAO had an area under ROC curve (AUC) for the diagnosis of SSc of 0.618 (95%CI 0.539- 0.697); with Se=0.373 (0.304-0.449) and Sp=0.862 (0.751-0.928). Fibrotic TS had an AUC of 0.561 (0.480-0.643); with Se=0.139 (0.094-0.199) and Sp=0.983 (0.909-0.997). The US-SSc pattern had a AUC of 0.641 (0.563- 0.695), with Se=0.440 (0.367-0.516) and Sp=0.845 (0.731-0.916). A scoring system including these US parameters and items from ACR/EULAR classification criteria had an AUC of 0.979 (0.962-0.996)) and allows the substitution of capillaroscopy by US parameters with similar performances. CONCLUSIONS: The use of hand US parameters may help to refine the diagnostic strategy of SSc and their inclusion in a combined diagnostic approach could be discussed.


Assuntos
Esclerodermia Localizada , Escleroderma Sistêmico , Humanos , Angioscopia Microscópica , Artéria Ulnar , Ultrassonografia
6.
Dermatol Online J ; 26(6)2020 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-32815688

RESUMO

Calcinosis cutis, although common in systemic sclerosis, has been rarely reported in patients with morphea. We describe four patients with calcinosis cutis arising within morphea plaques, discuss their treatments and outcomes, and review previously published cases. Current management recommendations for concomitant morphea and dystrophic calcinosis cutis are based on limited data and expert opinion, which has primarily focused on reduction of active inflammation and reduction of symptoms related to calcinosis or ulceration. In most cases, no improvement of calcinosis was noted. The use of intralesional corticosteroids to active lesions in conjunction with systemic treatment, including methotrexate when indicated, appear promising treatments to halt progression of the disease. Surgical excision seems to be the most definitive treatment for calcinosis affecting morphea plaques, but the current literature lacks details regarding disease recurrence following operative management.


Assuntos
Calcinose/etiologia , Esclerodermia Localizada/complicações , Dermatopatias/patologia , Pele/patologia , Corticosteroides/uso terapêutico , Calcinose/patologia , Calcinose/cirurgia , Criança , Feminino , Humanos , Injeções Intralesionais , Metotrexato/uso terapêutico , Pessoa de Meia-Idade , Esclerodermia Localizada/tratamento farmacológico , Esclerodermia Localizada/patologia , Dermatopatias/terapia
7.
Dermatol Online J ; 26(6)2020 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-32815694

RESUMO

Bullous morphea is a rare variant of localized scleroderma characterized by occasional intermittent blisters. Lichen sclerosus is a chronic inflammatory disease. The coexistence of morphea and lichen sclerosus has been reported in different sites in the same patient and more rarely in the same lesion. We report the case of a 54-year-old woman with an atypical presentation of bullous morphea and some histological features of lichen sclerosus. She presented with a 5-year history of an ulcerated plaque, with a sclerotic and atrophic center and indurated budding margins, localized on the lumbar back. Initially the diagnosis of a squamous cell carcinoma was suggested. A skin biopsy confirmed the diagnosis of bullous morphea and showed some histological features of lichen sclerosus. Topical betamethasone and silicone gel ointment were prescribed leading to complete healing of the ulceration within five months. Our case is unusual because of the atypical clinical presentation, the histological aspect combining signs of bullous morphea and lichen sclerosus, and the favorable results with the use of local corticotherapy and silicone gel.


Assuntos
Esclerodermia Localizada/patologia , Géis de Silicone/administração & dosagem , Administração Tópica , Corticosteroides/administração & dosagem , Feminino , Humanos , Pessoa de Meia-Idade , Esclerodermia Localizada/tratamento farmacológico
8.
J Am Acad Orthop Surg ; 28(16): e686-e695, 2020 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-32769717

RESUMO

Scleroderma is derived from Latin meaning hard skin. It is an uncommon, noninflammatory connective tissue disorder characterized by increased fibrosis of the skin and in certain variants, multiple other organ systems. Scleroderma involves a spectrum of pathologic changes and anatomic involvement. It can be divided into localized and systemic scleroderma. Hand involvement is common and can include calcium deposits within the soft tissues, digital ischemia, and joint contracture. Nonsurgical management consists of lifestyle modifications, biofeedback, therapy for digital stiffness/contracture, and various pharmacologic medications. When nonsurgical measures are unsuccessful, certain surgical options may be indicated, each with their inherent advantages and pitfalls. Patients with scleroderma who are undergoing surgical intervention pose unique difficulties because of their poorly vascularized tissue and deficient soft-tissue envelopes, thus increasing their susceptibility to wound healing complications and infection. Some subgroups of patients are frequently systemically ill, and specific perioperative measures should be considered to reduce their surgical risk. The spectrum of hand manifestations seen in patients with scleroderma will be reviewed with the focus on evaluation and management.


Assuntos
Mãos , Procedimentos Ortopédicos/métodos , Esclerodermia Localizada/cirurgia , Escleroderma Sistêmico/cirurgia , Calcinose , Mãos/patologia , Mãos/cirurgia , Humanos , Comunicação Interdisciplinar , Equipe de Assistência ao Paciente , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/patologia , Esclerodermia Localizada/terapia , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/terapia
10.
J Assoc Physicians India ; 68(4): 68-70, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32610852

RESUMO

Systemic sclerosis (SSc) is a connective tissue disorder of unknown aetiology. A small subset (10%) of patients with limited systemic sclerosis have all other features of the disease without any skin involvement and is known as systemic sclerosis sine scleroderma (ssSSc). Severe Critical Limb Ischaemia is rare in sine scleroderma.The present case showed severe critical limb ischaemia with severe PAH,Esophageal dysmotility,Glomerulonephritis(a rare association) with hypertension. Although skin thickening is considered as a hallmark of systemic sclerosis, there should be a high index of clinical suspicion in patients presenting with possible manifestations of systemic sclerosis without sclerodermatous cutaneous involvement because early diagnosis and treatment can reduce the morbidity and mortality in it.


Assuntos
Escleroderma Sistêmico , Humanos , Esclerodermia Limitada , Esclerodermia Localizada
12.
Hautarzt ; 71(10): 802-804, 2020 Oct.
Artigo em Alemão | MEDLINE | ID: mdl-32533203

RESUMO

We present a rare case of a 4-year-old girl suffering from a sclerotic lesion on the forehead as well as uveitis and epileptic seizures. The patient was diagnosed with linear scleroderma "en coup de sabre" (LSECDS). Faced with no gold standard for treatment of LSECDS with severe extracutaneous manifestations, the case was intensively discussed within an interdisciplinary team setting and successfully treated.


Assuntos
Esclerodermia Localizada/diagnóstico , Dermatopatias/complicações , Pré-Escolar , Eletroencefalografia , Feminino , Testa , Humanos , Esclerodermia Localizada/tratamento farmacológico , Esteroides/uso terapêutico , Resultado do Tratamento
14.
J Dtsch Dermatol Ges ; 18(7): 669-673, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32364667

RESUMO

Scleroderma is a heterogeneous group of fibrosing connective tissue disorders of unknown etiology. Morphea is a localized form of scleroderma that occasionally leads to chronic erosions and ulcerations of the skin. Fibrosis, inflammation and chronic ulcerations may eventually promote skin neoplasms; morphea is therefore a rare but established risk factor for cutaneous squamous cell carcinoma (cSCC). We present a review of 16 scleroderma patients: 15 case reports from the literature (identified by a PubMed search) and one case from our clinic of a patient who had developed cSCC, and we discuss potential underlying mechanisms. Statistical analysis revealed that the lower extremities were the body site most commonly affected by cSCC in these scleroderma patients. The mean time interval between the onset of scleroderma and the development of cSCC was ten to twenty years. In patients with morphea, we recommend checking for skin tumors during follow-up examinations as well as a careful risk-benefit analysis when considering the application of immunosuppressants or phototherapy in view of their potential carcinogenic side effects.


Assuntos
Carcinoma de Células Escamosas/etiologia , Esclerodermia Localizada/complicações , Neoplasias Cutâneas/etiologia , Adolescente , Adulto , Idade de Início , Idoso , Carcinoma de Células Escamosas/patologia , Feminino , Humanos , Imunossupressores/efeitos adversos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Fototerapia/efeitos adversos , Fatores de Risco , Esclerodermia Localizada/patologia , Esclerodermia Localizada/terapia , Neoplasias Cutâneas/patologia , Adulto Jovem
15.
Clin Exp Dermatol ; 45(6): 673-678, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32472964

RESUMO

Morphoea, also known as localized scleroderma, is a debilitating fibrosing disorder of uncertain aetiology, affecting the skin and subcutaneous tissues. Paediatric-onset disease is not uncommon and is associated with frequent relapses. The disease has complex pathogenetic mechanisms and multiple clinical subtypes, and affects children of all ages. Recent research has focused on elucidating the disease pathophysiology and identifying measures of disease activity. We performed a literature search on PubMed, MEDLINE and Google Scholar, using keywords such as 'pediatric morphea', 'juvenile localised scleroderma' and 'juvenile systemic sclerosis'. Relevant studies, including randomized trials, reviews of standard current guidelines and original research articles, were selected, and results were analysed before being summarized. In the first of this two-part review, we provide a bird's-eye view of the current literature concerning the epidemiology, aetiopathogenesis and clinical classification of paediatric morphoea; in Part 2, we review the diagnosis, markers of disease activity, management and natural history.


Assuntos
Esclerodermia Localizada , Criança , Humanos , Esclerodermia Localizada/classificação , Esclerodermia Localizada/epidemiologia , Esclerodermia Localizada/etiologia , Escleroderma Sistêmico/classificação , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/etiologia
16.
Clin Exp Dermatol ; 45(6): 679-684, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32449205

RESUMO

Paediatric morphoea is a debilitating fibrosing disorder of uncertain aetiology, affecting the skin and subcutaneous tissues. Defining optimum management strategies in paediatric morphoea remains an ongoing challenge, owing to the varied presentations and a relative paucity of paediatric-specific studies. We performed a literature search on PubMed, MEDLINE and Google Scholar, using keywords such as 'pediatric morphea', 'juvenile localised scleroderma' and 'juvenile systemic sclerosis'. Relevant studies, including randomized trials, reviews of standard current guidelines and original research articles, were selected and results analysed before summarizing them. In Part 1 of this review, we described the epidemiology, aetiopathogenesis and clinical classification; in this part, we discuss the diagnosis, markers of disease activity, management and natural history in paediatric morphoea.


Assuntos
Corticosteroides/uso terapêutico , Fototerapia , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/terapia , Biomarcadores , Criança , Humanos , Metotrexato/uso terapêutico , Ácido Micofenólico/uso terapêutico , Esclerodermia Localizada/patologia , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/terapia
17.
Health Qual Life Outcomes ; 18(1): 133, 2020 May 12.
Artigo em Inglês | MEDLINE | ID: mdl-32398135

RESUMO

BACKGROUND: Localized Scleroderma (LoS) is an autoimmune connective tissue disease that affects skin and less commonly subcutaneous tissues. The illness occurs in children and adults, and may have a serious impact on health-related quality of life (HRQoL). The goal of this study was to explore what factors might deteriorate scores on HRQoL measures in adult LoS patients. METHODS: Detailed information on the demographic and clinical features of LoS patients was collected. The HRQoL was assessed using Skindex-29 and Short form 36 (SF-36) questionnaires. RESULTS: Thirty three women and seven men with LoS were enrolled. Female gender negatively influenced scores for the emotion subscale of Skindex-29. Multiple-factor linear regression analysis confirmed, as with single-factor analysis, that the causes of low SF-36 physical component score (PCS) were subjective symptoms (pruritus, pain, paresthesia), musculoskeletal manifestations and older age at the time of survey, while rural area of residence negatively affected the SF-36 mental component score. Additionally, single-factor analysis showed that the SF-36 PCS was related to the LoS cutaneous assessment tool (LoSCAT) summary score. CONCLUSIONS: Apart from a clinical presentations, sociodemographic characteristics, including older age, female gender and living conditions, may impair HRQoL of LoS patients. Further studies that will examine the role of these factors for physical and mental functioning of adults with LoS are needed.


Assuntos
Qualidade de Vida , Esclerodermia Localizada/psicologia , Adulto , Fatores Etários , Estudos Transversais , Feminino , Humanos , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Inquéritos e Questionários
19.
Reumatol. clín. (Barc.) ; 16(2,pt.2): 180-182, mar.-abr. 2020. ilus
Artigo em Espanhol | IBECS | ID: ibc-194345

RESUMO

La psoriasis es una frecuente dermatosis inflamatoria que puede asociarse a diversas enfermedades. Estudios recientes señalan que la presencia de enfermedades autoinmunes es mayor, pero es rara la asociación con enfermedades del tejido conectivo. La coexistencia con lupus es infrecuente. Por otra parte, la morfea raramente se ha reportado en pacientes con lupus o psoriasis. Presentamos a una paciente con lupus cutáneo y morfea profunda que posteriormente desarrolló psoriasis, con excelente respuesta a metotrexato y revisamos la literatura


Psoriasis is a common inflammatory dermatosis that may be associated with a number of diseases. Recent studies provide evidence that there is a greater frequency of autoimmune diseases, but association with autoimmune connective tissue diseases is uncommon. The coexistence of psoriasis and lupus erythematosus is rare. Besides, the occurrence of morphea has rarely been reported in patients with lupus or psoriasis. We report a woman with cutaneous lupus and morphea profunda associated with psoriasis, with an excellent response to methotrexate, and review the literature


Assuntos
Humanos , Feminino , Idoso , Lúpus Eritematoso Cutâneo/diagnóstico , Lúpus Eritematoso Cutâneo/patologia , Esclerodermia Localizada/patologia , Psoríase/patologia , Couro Cabeludo/patologia , Lúpus Eritematoso Cutâneo/tratamento farmacológico , Esclerodermia Localizada/tratamento farmacológico , Psoríase/tratamento farmacológico , Autoimunidade , Prednisona/administração & dosagem , Corticosteroides/administração & dosagem , Administração Tópica , Couro Cabeludo/efeitos dos fármacos , Biópsia , Psoríase/etiologia
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