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Dermatomyositis (CUI C0011633) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C0011633

NCI Thesaurus Code: C26744  (see NCI Thesaurus info)

Semantic Type: Disease or Syndrome

NCIt Definition: Inflammation of the skin and muscle.

GARD Definition: Dermatomyositis is one of a group of acquired muscle diseases called inflammatory myopathies (disorder of muscle tissue or muscles), which are characterized by chronic muscle inflammation accompanied by muscle weakness. The cardinal symptom is a skin rash that precedes or accompanies progressive muscle weakness.  Dermatomyositis may occur at any age, but is most common in adults in their late 40s to early 60s, or children between 5 and 15 years of age. There is no cure for dermatomyositis, but the symptoms can be treated. Options include medication, physical therapy, exercise, heat therapy (including microwave and ultrasound), orthotics and assistive devices, and rest. The cause of dermatomyositis is unknown.

- this information is from GARD/ORDR/NCATS.

CSP Definition: progressive condition characterized by symmetric proximal muscular weakness with elevated serum levels of muscle enzymes and a skin rash, typically a purplish-red erythema on the face, and edema of the eyelids and periorbital tissue; affected muscle tissue shows degeneration of fibers with a chronic inflammatory reaction; occurs in children and adults, and in the latter may be associated with visceral cancer or other disorders of connective tissue.

MSH Definition: A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. The illness occurs with approximately equal frequency in children and adults. The skin lesions usually take the form of a purplish rash (or less often an exfoliative dermatitis) involving the nose, cheeks, forehead, upper trunk, and arms. The disease is associated with a complement mediated intramuscular microangiopathy, leading to loss of capillaries, muscle ischemia, muscle-fiber necrosis, and perifascicular atrophy. The childhood form of this disease tends to evolve into a systemic vasculitis. Dermatomyositis may occur in association with malignant neoplasms. (From Adams et al., Principles of Neurology, 6th ed, pp1405-6)

Synonyms & Abbreviations: (see Synonym Details)
DERMATOMUCOSOMYOSITIS
Dermatomyositides
Dermatomyositis (disorder)
Dermatomyositis [Disease/Finding]
dermatomyositis
Dermatopolymyositides
Dermatopolymyositis, unsp, organ involvement unspecified
Dermatopolymyositis, unspecified, organ involvement unspecified
Dermatopolymyositis, unspecified
Dermatopolymyositis
DM - Dermatomyositis
DM
Polymyositis Dermatomyositis
Polymyositis with skin involvement
Polymyositis-Dermatomyositides
Polymyositis-Dermatomyositis
POLYMYOSITIS/DERMATOMYOSITIS
WAGNER-UNVERRICHT SYNDROME

External Source Codes: 
NCI Thesaurus Code C26744 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
AN coord IM with PARANEOPLASTIC SYNDROMES (IM) if pertinent MSH
AQL BL CF CI CL CN CO DG DH DI DT EC EH EM EN EP ET GE HI IM ME MI MO NU PA PC PP PS PX RH RT SU TH UR VE VI MSH
CASE_SIGNIFICANCE_ID 900000000000017005 SNOMEDCT_US
CASE_SIGNIFICANCE_ID 900000000000448009 SNOMEDCT_US
Contributing_Source FDA NCI
CTV3ID XUY09 SNOMEDCT_US
DATE_CREATED 2015-01-22T00:04:00 GARD
DC 1 MSH
DEFINITION_STATUS_ID 900000000000074008 SNOMEDCT_US
DID 1982-9738 CSP
DISEASE_IDENTIFIER_ID 31444 GARD
DISEASE_IDENTIFIER_ID 31445 GARD
DX 19660101 MSH
EFFECTIVE_TIME 20030731 SNOMEDCT_US
FDA_Table Patient Code (Appendix B) NCI
HAS_GARD_PAGE true GARD
IAN DEFAULT ICD10
ICE Poikilodermatomyositis; Polymyositis with skin involvement ICD9CM
IDENTIFIER_SOURCE Orp GARD
IDENTIFIER_TYPE_ID 1 GARD
IS_ACTIVE true GARD
IS_RARE true GARD
IS_SPANISH false GARD
LAST_REVIEW_DATE 2009-01-19T00:00:00 GARD
MDA 19990101 MSH
MESH_DEFINITION A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. The illness occurs with approximately equal frequency in children and adults. The skin lesions usually take the form of a purplish rash (or less often an exfoliative dermatitis) involving the nose, cheeks, forehead, upper trunk, and arms. The disease is associated with a complement mediated intramuscular microangiopathy, leading to loss of capillaries, muscle ischemia, muscle-fiber necrosis, and perifascicular atrophy. The childhood form of this disease tends to evolve into a systemic vasculitis. Dermatomyositis may occur in association with malignant neoplasms. (From Adams et al., Principles of Neurology, 6th ed, pp1405-6) NDFRT
MESH_DUI D003882 NDFRT
MESH_NAME Dermatomyositis NDFRT
MESH_UI M0006061 NDFRT
MMR 20150622 MSH
MN C05.651.594.819.500 MSH
MN C10.668.491.562.575.500 MSH
MN C17.300.250 MSH
MN C17.800.185 MSH
NDFRT_KIND DISEASE_KIND NDFRT
NUI N0000000946 NDFRT
ORDER_NO 17956 ICD10CM
ORDER_NO 17972 ICD10CM
ORDER_NO 17973 ICD10CM
PRIMARY_PATH 10012503$10010760$10014982$10040785$Dermatomyositis$Connective tissue disorders$Epidermal and dermal conditions$Skin and subcutaneous tissue disorders MDR
PRIMARY_SOC 10040785 MDR
PT_IN_VERSION 10.0 MDR
PT_IN_VERSION 10.1 MDR
PT_IN_VERSION 11.0 MDR
PT_IN_VERSION 11.1 MDR
PT_IN_VERSION 12.0 MDR
PT_IN_VERSION 12.1 MDR
PT_IN_VERSION 13.0 MDR
PT_IN_VERSION 13.1 MDR
PT_IN_VERSION 14.0 MDR
PT_IN_VERSION 14.1 MDR
PT_IN_VERSION 15.0 MDR
PT_IN_VERSION 15.1 MDR
PT_IN_VERSION 16.0 MDR
PT_IN_VERSION 16.1 MDR
PT_IN_VERSION 17.0 MDR
PT_IN_VERSION 17.1 MDR
PT_IN_VERSION 18.0 MDR
PT_IN_VERSION 18.1 MDR
PT_IN_VERSION 19.0 MDR
PT_IN_VERSION 19.1 MDR
PT_IN_VERSION 20.0 MDR
PT_IN_VERSION 20.1 MDR
PT_IN_VERSION 8.0 MDR
PT_IN_VERSION 8.1 MDR
PT_IN_VERSION 9.0 MDR
PT_IN_VERSION 9.1 MDR
RARE_DISEASE_URL http://rarediseases.info.nih.gov/gard/6263/dermatomyositis/resources/1 GARD
RXAUI 3145946 RXNORM
RXAUI 3145947 RXNORM
RXAUI 3145954 RXNORM
RXAUI 3222228 RXNORM
RXCUI 1023376 RXNORM
SNOMED_CID 396230008 NDFRT
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS M33.1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~M33.1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS M33.90 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF ADULT ONSET DERMATOMYOSITIS CHOOSE M33.10 | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF AGE AT ONSET OF CLINICAL FINDING ON OR AFTER 1.0 YEAR AND IF AGE AT ONSET OF CLINICAL FINDING BEFORE 18.0 YEARS CHOOSE M33.00 | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF CHILDHOOD TYPE DERMATOMYOSITIS CHOOSE M33.00 | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF DERMATOMYOSITIS SINE MYOSITIS CHOOSE M33.92 | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF DERMATOMYOSITIS WITH MALIGNANT DISEASE CHOOSE D49.9 | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF DERMATOMYOSITIS WITH MALIGNANT DISEASE CHOOSE M36.0 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF DRUG-INDUCED DERMATOMYOSITIS CHOOSE G72.0 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF DRUG-INDUCED DERMATOMYOSITIS CHOOSE T50.905? | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE | EPISODE OF CARE INFORMATION NEEDED | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT | POSSIBLE REQUIREMENT FOR AN EXTERNAL CAUSE CODE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF JUVENILE DERMATOMYOSITIS CO-OCCURRENT WITH RESPIRATORY INVOLVEMENT CHOOSE M33.01 | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF POIKILODERMATOMYOSITIS CHOOSE M33.10 | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF SCLERODERMATOMYOSITIS CHOOSE M33.10 | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~MAP SOURCE CONCEPT CANNOT BE CLASSIFIED WITH AVAILABLE DATA SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447638001 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447639009 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~10 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~3 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~4 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~5 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~6 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~7 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~8 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~9 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 1212005 | Childhood type dermatomyositis | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 238935002 | Dermatomyositis sine myositis | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 238936001 | Sclerodermatomyositis | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 239901009 | Dermatomyositis with malignant disease | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 311671000119100 | Juvenile dermatomyositis co-occurrent with respiratory involvement | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 402425006 | Adult onset dermatomyositis | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 403635000 | Drug-induced dermatomyositis | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 445518008 | Age at onset of clinical finding (observable entity) | >= 1.0 year AND IFA 445518008 | Age at onset of clinical finding (observable entity) | < 18.0 years SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 86365006 | Poikilodermatomyositis | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~OTHERWISE TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~D49.9 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~G72.0 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~M33.00 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~M33.01 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~M33.10 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~M33.90 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~M33.92 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~M36.0 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~T50.905? SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~ SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~XUY09 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
TERMUI T011553 MSH
TERMUI T372461 MSH
TERMUI T372462 MSH
TH NLM (1966) MSH
TH NLM (2000) MSH
TYPE_ID 900000000000003001 SNOMEDCT_US
TYPE_ID 900000000000013009 SNOMEDCT_US

Additional Concept Data:  (none)

URL to Bookmark: https://ncim-stage.nci.nih.gov/ncimbrowser/ConceptReport.jsp?dictionary=NCI Metathesaurus&code=C0011633

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