Scleroderma and Related Disorders

2017 ◽  
Author(s):  
Kristine Phillips

Scleroderma spectrum diseases are a heterogeneous group of disorders that are distinguished by abnormalities of the connective tissue in the skin and, in some cases, other organs. Each disorder may be characterized by the extent of cutaneous and internal involvement, as well as histopathologic features of skin biopsy. Scleroderma spectrum diseases include systemic scleroderma, localized scleroderma, and eosinophilic fasciitis. This chapter reviews the classification, epidemiology, etiology, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, outcome measures, management, and clinical course of scleroderma as well as the definition and classification, etiology/genetics, differential diagnosis, and treatment of localized scleroderma. Also discussed are the definition and classification, epidemiology, etiology/genetics/pathogenesis, diagnosis, differential diagnosis, and treatment of eosinophilic fasciitis. Tables review the classification of—and antinuclear antibodies in—scleroderma as well as the key assessments and interventions in scleroderma management. Figures illustrate the disease's presentation and clinical manifestations, including several images of scleroderma of the hands; face, palmar, and buccal telangiectasias in a patient with scleroderma; a radiograph demonstrating calcinosis of the elbow; Raynaud’s phenomenon; high-resolution computed tomographic images of diffuse cutaneous scleroderma, scleroderma and severe pulmonary hypertension, and limited cutaneous scleroderma; plus an esophagram demonstrating hypomotility. This review contains 11 highly rendered figures, 3 tables, and 72 references.

2017 ◽  
Author(s):  
Kristine Phillips

Scleroderma spectrum diseases are a heterogeneous group of disorders that are distinguished by abnormalities of the connective tissue in the skin and, in some cases, other organs. Each disorder may be characterized by the extent of cutaneous and internal involvement, as well as histopathologic features of skin biopsy. Scleroderma spectrum diseases include systemic scleroderma, localized scleroderma, and eosinophilic fasciitis. This chapter reviews the classification, epidemiology, etiology, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, outcome measures, management, and clinical course of scleroderma as well as the definition and classification, etiology/genetics, differential diagnosis, and treatment of localized scleroderma. Also discussed are the definition and classification, epidemiology, etiology/genetics/pathogenesis, diagnosis, differential diagnosis, and treatment of eosinophilic fasciitis. Tables review the classification of—and antinuclear antibodies in—scleroderma as well as the key assessments and interventions in scleroderma management. Figures illustrate the disease's presentation and clinical manifestations, including several images of scleroderma of the hands; face, palmar, and buccal telangiectasias in a patient with scleroderma; a radiograph demonstrating calcinosis of the elbow; Raynaud’s phenomenon; high-resolution computed tomographic images of diffuse cutaneous scleroderma, scleroderma and severe pulmonary hypertension, and limited cutaneous scleroderma; plus an esophagram demonstrating hypomotility. This review contains 11 highly rendered figures, 3 tables, and 72 references.


2018 ◽  
Author(s):  
Kristine Phillips

Scleroderma spectrum diseases are a heterogeneous group of disorders that are distinguished by abnormalities of the connective tissue in the skin and, in some cases, other organs. Each disorder may be characterized by the extent of cutaneous and internal involvement, as well as histopathologic features of skin biopsy. Scleroderma spectrum diseases include systemic scleroderma, localized scleroderma, and eosinophilic fasciitis. This chapter reviews the classification, epidemiology, etiology, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, outcome measures, management, and clinical course of scleroderma as well as the definition and classification, etiology/genetics, differential diagnosis, and treatment of localized scleroderma. Also discussed are the definition and classification, epidemiology, etiology/genetics/pathogenesis, diagnosis, differential diagnosis, and treatment of eosinophilic fasciitis. Tables review the classification of—and antinuclear antibodies in—scleroderma as well as the key assessments and interventions in scleroderma management. Figures illustrate the disease's presentation and clinical manifestations, including several images of scleroderma of the hands; face, palmar, and buccal telangiectasias in a patient with scleroderma; a radiograph demonstrating calcinosis of the elbow; Raynaud’s phenomenon; high-resolution computed tomographic images of diffuse cutaneous scleroderma, scleroderma and severe pulmonary hypertension, and limited cutaneous scleroderma; plus an esophagram demonstrating hypomotility. This review contains 11 highly rendered figures, 3 tables, and 72 references.


2019 ◽  
pp. 101-107
Author(s):  
L. A. Poroshina ◽  
E. I. Rublevskaya ◽  
S. L. Achinovich

The article presents up-to-date literary data on the etiopathogenesis, prevalence of lichen sclerosus, defines the clinical manifestations and classification of the disease, and considers the issues of its diagnosis and treatment. The article also describes 3 clinical cases of genital lesions in female patients with localized scleroderma. In our opinion, lichen sclerosus is one of the forms of localized scleroderma.


2015 ◽  
Vol 105 (3) ◽  
pp. 260-263 ◽  
Author(s):  
Erkam Komurcu ◽  
Burak Kaymaz ◽  
Umut Hatay Golge ◽  
Ferdi Goksel ◽  
Mustafa Resorlu ◽  
...  

A 53-year-old man presented to the orthopedic outpatient clinic with pain and swelling in the right heel without any trauma. On physical examination and radiologic assessment, a lesion with calcification and peripheral sclerosis was detected in the medullary cavity of the calcaneus, and computed tomographic images revealed cortical thinning adjacent to the lesion. Magnetic resonance images showed a 23 × 19-mm lesion. Tru-Cut biopsy, performed to clarify the diagnosis, revealed an enchondroma. As a definitive treatment, curettage of the lesion and grafting of the cavity was performed. Although enchondromas are common pathologic abnormalities of the skeleton and are usually asymptomatic, atypical localization, such as the calcaneus, and atypical clinical manifestations, such as heel pain, should also be kept in mind.


Neurosurgery ◽  
1983 ◽  
Vol 13 (4) ◽  
pp. 345-350 ◽  
Author(s):  
Timothy W. Phillips ◽  
John E. McGillicuddy ◽  
Julian T. Hoff ◽  
Joseph Latack

Abstract The occurrence of a brain stem neoplasm in three adults previously treated for an adult Arnold-Chiari malformation is reported. The diagnosis of adult Arnold-Chiari malformation had been confirmed by angiography and computed tomographic scanning. All patients were treated with a combination of ventricular shunts and posterior fossa decompression. The diagnosis of brain stem neoplasm was not made until late in the clinical course, and all patients died within 2 years of the onset of symptoms.


2020 ◽  
Vol 1 (1) ◽  
Author(s):  
Hina Handa

Oral mucosal lesions are a broad group of lesions which are located in the soft tissues of the oral cavity identified by its specific etiology, clinical manifestations, differential diagnosis and treatment. White lesions of the oral cavity constitute a rather common group of lesions that are encountered during routine clinical dental practice. The process of clinical diagnosis and treatment planning is of great concern to the patient as it determines the prognosis. There should be in-depth knowledge to the consultant about the varied clinical features, etiology of the disease and various treatment plans. Clinical diagnostic skills and ideal judgment forms the key to successful management of white lesions of the oral cavity This review will discuss many of the most common intraoral white lesions including their clinical presentation, how to make an accurate diagnosis


Vestnik ◽  
2021 ◽  
pp. 73-77
Author(s):  
Г.Ж. Бодыков ◽  
Г.Н. Балмагамбетова ◽  
С.А. Лисогор ◽  
В.М. Шмонин

Синдром Казабаха - Мерритта в детской практике встречается крайне редко. Клинические проявления многообразны, что затрудняет своевременную диагностику и лечение данного заболевания. Цель: привлечь внимание неонатологов и педиатров к своевременной диагностике редких гематологических синдромов. Материалы и методы. Проведено расширенное обследование ребенка и тщательная дифференциальная диагностика с другими сосудистыми аномалиями. Результаты. На основании оценки анамнеза, клинико - лабораторных и инструментальных методов обследования установлен диагноз СКМ. Выводы. Постановка диагноза СКМ требует проведения расширенного обследования ребенка и тщательной дифференцировки с другими сосудистыми аномалиями. Kazabach-Merritt syndrome is extremely rare in pediatric practice. Clinical manifestations are diverse- and this fact complicates the early diagnosis and treatment of the disease.Purpose: to draw the attention of neonatologists and pediatricians to the early diagnosis of rare hematological syndromes.Materials and methods. An extended examination of the child and a thorough differential diagnosis with other vascular anomalies were carried out. Results. The KMS was diagnosed on the base of: the assessment of the anamnesis, clinical - laboratory and instrumental examination methods.Conclusion. The diagnosis of KMS requires an extended examination of the child and careful differentiation of other vascular anomalies.


1983 ◽  
Vol 59 (4) ◽  
pp. 596-600 ◽  
Author(s):  
Mark C. Glazier ◽  
Richard B. Kohler ◽  
Robert L. Campbell

✓ Legionella pneumophila postoperative pneumonia may be an important cause of morbidity and mortality in selected surgical patients. This report presents five postoperative neurosurgical patients in whom the diagnosis of Legionnaires' disease was made. Their clinical course and treatment are presented. Clinical manifestations, methods of diagnosis, and treatment of L. pneumophila pneumonia are discussed. It is pointed out that neurosurgical patients who have received high doses of corticosteroids and who develop nosocomial postoperative pneumonias should be suspected of having Legionnaires' disease.


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