Biomaterial Database

Systemic sclerosis and localized scleroderma in childhood. 2008

Francesco Zulian

Department of Pediatrics, Pediatric Rheumatology Unit, University of Padova, Via Giustiniani 3, 35128 Padova, Italy. zulian@pediatria.unipd.it

Juvenile scleroderma syndromes, including the systemic and the localized varieties, represent the third most frequent chronic rheumatic conditions in pediatric rheumatology practice. In children, systemic sclerosis shows a significantly less frequent involvement of all organs, a higher prevalence of arthritis and myositis, and a better outcome than in adults. Recently, new classification criteria were proposed, which help improve patient care by enabling earlier, more definite diagnoses and by standardizing the conduct of clinical trials. Localized scleroderma is the more frequent subtype of scleroderma in childhood. It comprises a group of distinct conditions that involve mainly the skin and subcutaneous tissues. They range from small plaques of fibrosis involving only the skin to diseases causing significant functional deformity with various extracutaneous features.

UI	MeSH Term	Description	Entries
D007166	Immunosuppressive Agents	Agents that suppress immune function by one of several mechanisms of action. Classical cytotoxic immunosuppressants act by inhibiting DNA synthesis. Others may act through activation of T-CELLS or by inhibiting the activation of HELPER CELLS. While immunosuppression has been brought about in the past primarily to prevent rejection of transplanted organs, new applications involving mediation of the effects of INTERLEUKINS and other CYTOKINES are emerging.	Immunosuppressant,Immunosuppressive Agent,Immunosuppressants,Agent, Immunosuppressive,Agents, Immunosuppressive
D010789	Phototherapy	Treatment of disease by exposure to light, especially by variously concentrated light rays or specific wavelengths.	Blue Light Therapy,Blue-light Therapy,Light Therapy,Photoradiation Therapy,Red Light Phototherapy,Therapy, Photoradiation,Blue Light Therapies,Blue-light Therapies,Light Phototherapies, Red,Light Phototherapy, Red,Light Therapies,Light Therapies, Blue,Light Therapy, Blue,Photoradiation Therapies,Phototherapies,Phototherapies, Red Light,Phototherapy, Red Light,Red Light Phototherapies,Therapies, Blue Light,Therapies, Blue-light,Therapies, Light,Therapies, Photoradiation,Therapy, Blue Light,Therapy, Blue-light,Therapy, Light
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D002986	Clinical Trials as Topic	Works about pre-planned studies of the safety, efficacy, or optimum dosage schedule (if appropriate) of one or more diagnostic, therapeutic, or prophylactic drugs, devices, or techniques selected according to predetermined criteria of eligibility and observed for predefined evidence of favorable and unfavorable effects. This concept includes clinical trials conducted both in the U.S. and in other countries.	Clinical Trial as Topic
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D012594	Scleroderma, Localized	A term used to describe a variety of localized asymmetrical SKIN thickening that is similar to those of SYSTEMIC SCLERODERMA but without the disease features in the multiple internal organs and BLOOD VESSELS. Lesions may be characterized as patches or plaques (morphea), bands (linear), or nodules.	Dermatosclerosis,Morphea,Scleroderma, Circumscribed,Frontal Linear Scleroderma en Coup de Sabre,Linear Scleroderma,Scleroderma, Linear,Circumscribed Scleroderma,Localized Scleroderma,Morpheas,Sclerodermas, Localized
D012595	Scleroderma, Systemic	A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA.	Sclerosis, Systemic,Systemic Scleroderma,Systemic Sclerosis