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Childhood type dermatomyositis

MedGen UID:
120486
•Concept ID:
C0263666
•
Disease or Syndrome
Synonym: Juvenile dermatomyositis
SNOMED CT: Juvenile dermatomyositis (1212005); Childhood type dermatomyositis (1212005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0008054
Orphanet: ORPHA93672

Definition

An early-onset form of dermatomyositis (DM), a systemic, autoimmune inflammatory muscle disorder with vasculopathy, characterized by proximal and symmetrical muscle weakness, evocative skin lesions, and systemic manifestations. Vasculopathy occurs in the skin, muscle (mainly in the perifascicular area), and sometimes in the intestinal tissue. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood type dermatomyositis

Professional guidelines

PubMed

Childhood Arthritis and Rheumatology Research Alliance consensus clinical treatment plans for juvenile dermatomyositis with skin predominant disease.
Kim S, Kahn P, Robinson AB, Lang B, Shulman A, Oberle EJ, Schikler K, Curran ML, Barillas-Arias L, Spencer CH, Rider LG, Huber AM
Pediatr Rheumatol Online J 2017 Jan 11;15(1):1. doi: 10.1186/s12969-016-0134-0. PMID: 28077146Free PMC Article

Recent clinical studies

Therapy

Dermatomyositis treated with high-dose intravenous immunoglobulins and associated with panniculitis.
Sabroe RA, Wallington TB, Kennedy CT
Clin Exp Dermatol 1995 Mar;20(2):164-7. doi: 10.1111/j.1365-2230.1995.tb02675.x. PMID: 8565257

Clinical prediction guides

Childhood-type myositis and linear scleroderma.
Miike T, Ohtani Y, Hattori S, Ono T, Kageshita T, Matsuda I
Neurology 1983 Jul;33(7):928-30. doi: 10.1212/wnl.33.7.928. PMID: 6683378

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

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    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

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