Interstitial Lung disease In Childhood Rheumatic Disorders
Lourdes R. Laraya-Cuasay, Walter T. Hughes in Interstitial Lung Diseases in Children, 2019
Scleroderma is a rheumatic disease which occurs both in adults and children in systemic and localized forms. Morphea and linear scleroderma are localized forms of disease. It is the generalized disease, progressive systemic sclerosis (PSS), which is associated with severe pulmonary involvement. PSS is characterized by the appearance of Raynaud’s phenomenon, skin thickening and tightness, articular disability and involvement of heart, intestines, kidneys and lungs. Singsen et al.,2 following at least 13 children with PSS, found no significant differences between childhood and adult PSS. The incidence of PSS in the general population is approximately five cases per million people per year.22 In children, onset has been noted as early as 3 years. The female to male ratio is 6:1.4
Connective Tissue Disorders
Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang in Roxburgh's Common Skin Diseases, 2022
Management: There is no single evidence-based treatment regimen that is superior for all cases. Topical corticosteroids are the main treatment for plaque-like morphea. Topical tacrolimus 0.1% or calcipotriene are alternative choices. Active extensive morphea or generalized morphea are treated with methotrexate, combination therapy of systemic steroids and methotrexate, or with phototherapy with ultraviolet A1 (UVA1) or narrowband UVB. Linear scleroderma of the face or extremities generally requires the combination of systemic corticosteroids and methotrexate to limit functional disability and/or aesthetic outcomes.
Scleroderma and associated complications
Biju Vasudevan, Rajesh Verma in Dermatological Emergencies, 2019
Scleroderma refers to a rare connective tissue disorder characterized by sclerosis of the skin and subcutaneous tissue along with variable visceral involvement. Broadly scleroderma is classified into two categories: systemic sclerosis which encompasses cutaneous sclerosis and visceral involvement and localized scleroderma or morphea in which disease is mainly limited to the skin and underlying tissues. Systemic sclerosis is associated with multiple systemic complications and can present as emergencies. This chapter highlights the manifestations, complications, and management of systemic sclerosis.
Immunologic underpinnings and treatment of morphea
Published in Expert Review of Clinical Immunology, 2022
Avery H. LaChance, Nathaniel Goldman, Bina Kassamali, Ruth Ann Vleugels
Clinically, morphea presents as focal round, ovoid, band-like, or linear plaques of sclerosis of the skin and, occasionally, subcutaneous tissue, which can extend to include the muscles, joints, and bone. Importantly, although morphea was initially coined ‘localized scleroderma’ and shares some overlapping pathophysiologic and histopathologic features with systemic sclerosis, these two disease states are distinct entities. In morphea there is a notable absence of the hallmark cutaneous features of systemic sclerosis including skin thickening of the fingers of the bilateral hands, fingertip lesions (such as digital tip ulcers and pitting scars), telangiectasias, Raynaud’s phenomenon, and abnormal nailfold capillaries [9]. In addition, systemic sclerosis is also characterized by multiple systemic manifestations including pulmonary, gastrointestinal, and renal disease, which are absent in patients with morphea. Morphea is predominantly a disease of the skin and subcutaneous tissue. However, extracutaneous features are seen in 20–70% of patients and can be associated with significant disease morbidity [10–12]. Extracutaneous features of morphea can include arthritis (even in areas without skin involvement) [13,14], joint contractures, limb length discrepancies, myositis, gait disturbance, as well as neurologic (i.e. headaches, seizures), ophthalmologic (i.e. Uveitis, optic scleritis), and dental complications [10]. A Total Morbidity Score (TMS) was recently proposed as a means of quantifying the extent extracutaneous involvement for patients with morphea and can be considered for use to capture the extent of extracutaneous disease burden and monitor for progression/response to treatment with time [15].
A case of linear scleroderma “en coup de sabre” with strabismus fixus in a child
Published in Strabismus, 2019
Pradhnya Sen, Easha Ramawat, Amit Mohan, Chintan Shah
Ramboer K et al.5 reported a case of linear scleroderma with MRI findings. They found that the eye was displaced laterally by a band like mass and superior oblique, SR and MR could not be identified, associated features of enophthalmos was also seen. The thickening of orbital muscles seen in their case was hypothesized secondary to orbital fat atrophy and fibrosis.
The role of exosome in autoimmune connective tissue disease
Published in Annals of Medicine, 2019
Tian Zhu, Yiman Wang, Hongzhong Jin, Li Li
Scleroderma is a chronic, systemic autoimmune disorder, which is characterized by excessive fibrosis caused by the abnormal growth of connective tissue supporting the skin and internal organs. Patients can exhibit vascular abnormalities as well as skin inflammation. The skin fibrosis is thought to be a result of excessive production of extracellular matrix proteins by dysfunctional dermal fibroblasts [31]. Two categories of scleroderma are known: systemic sclerosis (SSc), a chronic connective tissue disease characterized by inflammation, cutaneous sclerosis and visceral organ fibrosis, and localized scleroderma or morphea, which typically causes skin thickening and only occasionally affects the underlying muscle and tissue. Interestingly, increased expression of the exosomal markers, CD63, CD9 and CD81 has been observed in SSc dermal fibroblasts compared with normal fibroblasts, suggesting that increased levels of exosomes were present. The exosomes isolated from cultured SSc fibroblasts could stimulate type I collagen expression in normal fibroblasts. In addition, analysis of SSc fibroblast-derived exosomes revealed the dysregulation of collagen-related miRNA levels. Furthermore, serum exosome levels were significantly decreased in SSc patients compared with healthy control patients. Lower serum exosome levels positively correlated with the developments of vascular involvements such as skin ulcers or pitting scars. This could be the result of a disturbed transfer of exosomes from skin tissue to the bloodstream, with a wound healing delay caused by the additional down-regulation of collagen expression [32]. Skin ulcers are a frequent complication in SSc, which severely affects patients’ quality of life. Recently, new therapeutic strategies for skin ulcers such as topical negative pressure therapy and platelet-rich plasma have been developed. In addition, serum-derived exosomes have been demonstrated to aid the healing of skin ulcers. This provides a further example of exosomal involvement in disease pathogenesis and their therapeutic potential.
Related Knowledge Centers
- Autoantibody
- Collagen
- Fascia
- Panniculus
- Scleroderma
- Systemic Scleroderma
- Dermis
- Subcutaneous Tissue
- Skin Condition
- Physician