ABSTRACT
Objective:To discuss the clinical features, diagnosis and treatment of linear scleroderma (LS).Methods:A case of LS diagnosed in the Second Hospital of Shandong University in October 20, 2020, was reported and the clinical features and pathological documentation of the disease reported in the literature were reviewed.Results:A 24-year-old woman presented cicatricial alopecia on the left frontoparietal area and facial atrophy for about 10 years. Two years before, she began to suffer ptosis and neurological complaints. Clinical features of different stages of the disease are presented. All 15 patients reported in the literature were analyzed, with a median of 22 years and a male to female ratio of 9∶6. There were 4 cases of linear scleroderma with ipsilateral drooping eyelids and lateral contraction, 3 cases of linear scleroderma with demyelinating lesions, combined with lateral contraction, 3 cases of linear scleroderma combined with lateral atrophy, and 1 case of linear scleroderma with ipsilateral facial spasm. Two cases were with the chest sclerosing spot. Two cases of linear scleroderma were with epileptic seizure and white matter demyelination lesion. Six cases were treated with hormone, 2 cases were treated with methotrexate. One case was treated with both hormone and methotrexate. One case was treated with botulinum toxin. Three cases were treated with surgical correction of eyelid ptosis. One case was treated with ultraviolet A1 radiation phototherapy and 1 case was treated with vitamin therapy.Conclusions:Patients with scleroderma may have ipsilateral facial atrophy, blepharoptosis and facial spasm. Some patients involving the nervous system may have epilepsy and myelitis. And demyelinating lesions can be seen in magnetic resonance imaging. Localized scleroderma may develop into systemic scleroderma. Therefore, it is recommended to combine immunosuppressants as soon as possible to control the development of the disease if necessary.
ABSTRACT
ABSTRACT Introduction: Reynolds syndrome (RS) is an autoimmune disorder characterized by overlapping primary biliary cirrhosis (PBC) and limited cutaneous systemic sclerosis (lcSSc). Some published cases do not report pulmonary arterial hypertension (PAH), and diagnoses are usually based on clinical, immunological and histological findings, mainly focused on dermatologic features, on those associated with Sjogren's syndrome, or on an interesting presentation of malignant thymoma; only one case of reported PAH was found, but it was an image report. Case Presentation: This paper reports the case of a 75-year-old woman who presented with some of the features mentioned above, severe PAH, dyspnea for one month and two weeks of purulent expectoration, as well as generalized pruritus, nasal telangiectasias, Raynaud phenomenon, sclerodactyly, and high levels of alkaline phosphatase and transaminases. Pulmonary arterial hypertension was documented through a transthoracic echocardiogram, and inmunoflorecence reported mitochondrial and centromere patterns of antinuclear-antibodies. Consequently, RS was diagnosed and phosphodiesterase type-5 inhibitors were started for PAH treatment resulting in the improvement of dyspnea. Conclusion: Dyspnea could be caused by many conditions, but in the presence of clinical and physical findings, it suggests an autoimmune disorder. Scleroderma should be considered and, additionally, PAH should be investigated since it is present in up to 10% of patients, conferring a worse prognosis. Internists should keep in mind that these disorders may be associated with other autoimmune diseases.
Subject(s)
Humans , Raynaud Disease , Scleroderma, Localized , Hypertension, Pulmonary , Liver CirrhosisABSTRACT
Nodular sclerosis is a rare form of presentation related to both systemic and localized forms of scleroderma. We describe the case of a patient with nodular sclerosis in order to alert the medical community to recognize this entity.
Esclerose nodular é uma forma de apresentação cutânea rara relacionada tanto com a forma sistêmica como com a forma localizada da esclerodermia. Descrevemos aqui o caso de uma paciente esta forma nodular de esclerose, no sentido de alertar a comunidade médica para o reconhecimento dessa entidade.
Subject(s)
Adult , Female , Humans , Keloid/pathology , Scleroderma, Systemic/pathology , Biopsy , Skin/pathologyABSTRACT
FUNDAMENTOS: A esclerodermia é uma colagenose relativamente rara, cujo perfil de autoanticorpos está associado a diferentes manifestações clínicas. A prevalência de autoanticorpos na esclerodermia sofre influência racial e genética. OBJETIVO: Estudar a prevalência dos anticorpos anti-Scl-70, anticentrômero e anti-U1-RNP em pacientes com esclerodermia do sul do Brasil e verificar suas associações às manifestações clínicas. MÉTODOS: Estudo retrospectivo de análise de 66 pacientes com esclerodermia para presença de anti-Scl-70, anticentrômero (ACA) e anti-U1-RNP e de manifestações clínicas como: Raynaud, cicatrizes estelares, necrose digital, telangiectasias, calcinose, fibrose pulmonar, pleurites, pericardites, miocardiopatias, artralgias e artrites, grau de esclerose da pele, contraturas articulares e atritos de tendão, hipertensão pulmonar, manifestações esofágicas e crise renal. RESULTADOS: A prevalência do anti-Scl-70 foi de 17,8 por cento, a do ACA, de 33,3 por cento, e a do U1 RNP foi de 11,8 por cento. O anti-Scl-70 estava associado à forma difusa da doença (p=0,015), presença de miocardiopatias (p=0,016) e de cicatrizes estelares (p=0,05); o anticentrômero foi mais comum na forma limitada, embora sem significância estatística e mostrou-se protetor para as miocardiopatias (p=0,005). O anti-U1-RNP foi mais comum nas formas de superposição (p=0,0004). CONCLUSÃO: A prevalência e o perfil de associações clínicas dos autoanticorpos em esclerodermia de pacientes brasileiros assemelham-se aos da literatura mundial.
BACKGROUND: Scleroderma is a fairly rare connective tissue disease whose autoantibody profile is associated with different clinical manifestations. The prevalence of autoantibodies in scleroderma is influenced by race and genetics. OBJECTIVE: To study the prevalence of anti-Scl-70, anti-centromere (ACA) and anti-U1-RNP antibodies in patients with scleroderma in southern Brazil and verify their association with clinical manifestations of the disease. METHODS: A retrospective study involving 66 patients with scleroderma for the presence of anti-Scl-70, anti-centromere and anti-U1-RNP and of clinical manifestations such as Raynaud's phenomenon, digital micro scars, digital necrosis, telangiectasias, calcinosis, pulmonary fibrosis, pleuritis, pericarditis, cardiomyopathy, arthralgia and arthritis, skin sclerosis, joint contractures, tendon friction rubs, pulmonary hypertension, esophageal disorders and renal crisis. RESULTS: The prevalence of anti-Scl-70 was 17.8 percent , that of ACA was 33.3 percent and the prevalence of U1 RNP was 11.8 percent. Anti-Scl-70 was associated with the diffuse form of the disease (p = 0.015), presence of cardiomyopathies (p = 0.016) and digital micro scars (p = 0.05). Anti-centromere was more common in the limited form, although it was not statistically significant, and had a protective role associated with cardiomyopathies (p = 0.005). Anti-U1-RNP was more common in the overlap forms (p = 0.0004). CONCLUSION: The prevalence and profile of clinical associations of autoantibodies in Brazilian patients with scleroderma are similar to those found in the literature.
Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Autoantibodies/immunology , Nuclear Proteins/analysis , Scleroderma, Systemic/immunology , Antibodies, Antinuclear , Brazil/epidemiology , Retrospective Studies , Ribonucleoprotein, U1 Small Nuclear/immunology , Scleroderma, Systemic/epidemiologyABSTRACT
Isquemia é comum em esclerodermia sistêmica e é causada por vasoespasmo e trombose. As autoras analisaram a associação de eventos vasculares periféricos e anticorpos anticardiolipinas (aCl) em 54 esclerodérmicos. Em 100 por cento deles existia Raynaud; 59,2 por cento apresentaram cicatrizes estelares; 43,3 por cento, telangiectasias; 14,8 por cento, fenômenos tromboembólicos periféricos. ACl IgG foram positivos em 9,2 por cento dos casos e o IgM, em 7,4 por cento. Fenômenos embólicos periféricos estão associados a aCl IgG (p=0,03), não se encontrando associação com demais manifestações.
Ischemia is common in systemic scleroderma and it is caused by vasospasm and thrombosis. In the present study we analyzed the association of peripheral vascular events and anticardiolipin (aCl) antibodies in 54 patients suffering from systemic scleroderma. The results showed that 100 percent of the patients presented Raynaud; 59.2 percent presented digital micro scars; 43.3 percent, presented teleangiectasies and 14.8 percent, presented peripheral thromboembolism. ACl IgG were positive in 9.2 percent and IgM, in 7.4 percent. Peripheral tromboembolic phenomena had a positive association with aCl IgG (p=0.03). No other associations were found.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Antibodies, Anticardiolipin/blood , Peripheral Vascular Diseases/blood , Peripheral Vascular Diseases/etiology , Scleroderma, Systemic/blood , Scleroderma, Systemic/complicationsABSTRACT
FUNDAMENTOS: A capilaroscopia periungueal tem sido largamente utilizada para diagnóstico de esclerodermia sistêmica. Mais recentemente descobriu-se que também pode predizer o envolvimento de órgãos internos. OBJETIVO: Verificar se a capilaroscopia periungueal mostra correlação com a gravidade da esclerodermia sistêmica. MÉTODOS: Foram estudados a capilaroscopia periungueal de 14 pacientes com esclerodermia sistêmica quanto ao número médio de capilares dilatados e às áreas de desvascularização; a medida do envolvimento cutâneo pelo índice de Rodnan modificado; e o grau de gravidade da doença segundo escala proposta por Medsger e cols. RESULTADOS: Os resultados mostraram boa correlação do índice de desvascularização com o grau de gravidade da doença (p = 0.04). Não se encontrou correlação entre o aparecimento de dilatação capilar e o grau de gravidade da doença (p = 0.572). O grau de espessamento cutâneo não mostrou correspondência com o grau de dilatação capilar (p = 0.76), embora mostrasse tendência de associação com desvascularização (p = 0.07). CONCLUSÃO: Os autores concluem que a presença de desvascularização à capilaroscopia periungueal pode ser usada como elemento indicador de maior gravidade da esclerodermia sistêmica.
BACKGROUND: Nail fold capillaroscopy has been largely used in the diagnosis of systemic sclerosis. It has been recently discovered that this test is also able to predict internal organ damage in systemic sclerosis. OBJECTIVE: This study was carried out to verify whether nail fold capillaroscopy findings are correlated with disease severity. METHODS: We studied nail fold capillaroscopy findings regarding dilated and avascular areas from 14 patients with systemic sclerosis; degree of skin involvement by means of a modified Rodnan index; and disease severity with the scale proposed by Medsger et al. RESULTS: The results showed that the number of avascular areas has a good correlation with disease severity (p = 0.043), but not with the number of dilated capillaries (p = 0.57). The degree of cutaneous thickening was not related to the degree of capillary dilatation (p = 0.76), but tended to be associated with avascular areas (p = 0.07). CONCLUSION: The authors conclude that the number of avascular areas in nail fold capillaroscopy could be used as a sign of disease severity in systemic sclerosis.