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Steatocystoma multiplex (CUI C0259771) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C0259771

Semantic Type: Disease or Syndrome

MSH Definition: A disorder characterized by multiple, wide spread cutaneous cysts that often become inflamed and rupture. It is caused by the same mutations in the gene coding for KRT-17 that are causative mutations for Pachyonychia congenita, Type 2. Natal teeth involvement is sometimes associated with steatocystoma multiplex.

GARD Definition: Steatocystoma multiplex is a condition characterized by numerous skin cysts that tend to develop during puberty. Cysts most often develop on the chest, upper arms and face, but may develop all over the body in some cases. The cysts may become inflamed and cause scarring when they heal. The condition is thought to be caused by mutations in the KRT17 gene and appears to be inherited in an autosomal dominant manner. Some researchers have suggested that the condition may be a mild variant of pachyonychia congenita type 2. Treatment may include minor surgery to remove cysts and oral antibiotics or oral isotretinoin to reduce inflammation. - this information is from GARD/ORDR/NCATS.

HPO Definition: Multiple, localized or widespread, asymptomatic or inflammatory dermal cysts involving the pilosebaceous units. Lesions can appear anywhere on the body, but steatocystoma multiplex is more commonly involved with those areas of the skin with a high density of developed pilosebaceous units (e.g., the axilla, groin, neck, and proximal extremities). [HPO:probinson, pmid:20631281]

Synonyms & Abbreviations: (see Synonym Details)
Cyst, Multiple Sebaceous
Cysts, Multiple Sebaceous
Disseminated sebocystomatosis
Hereditary epidermal polycystic disease
Multiple sebaceous cysts
Multiple Sebaceous Cyst
Multiplex Steatocystomas
Multiplex Steatocystoma
Sebaceous Cyst, Multiple
Sebaceous Cysts, Multiple
Sebocystomatosis
Steatocystoma multiplex (disorder)
Steatocystoma Multiplex [Disease/Finding]
Steatocystoma Multiplex
Steatocystoma Multiplices
Steatocystoma, Multiplex
Steatocystomas, Multiplex

External Source Codes:  (none)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
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 TM UR VE VI MSH
CASE_SIGNIFICANCE_ID 900000000000448009 SNOMEDCT_US
CTV3ID X78S1 SNOMEDCT_US
DATE_CREATED 2012-07-27T01:45:14Z HPO
DATE_CREATED 2013-01-09T00:04:00 GARD
DATE_LAST_MODIFIED 2013-01-09T00:04:00 GARD
DC 1 MSH
DEFINITION_STATUS_ID 900000000000074008 SNOMEDCT_US
DISEASE_IDENTIFIER_ID 14859 GARD
DISEASE_IDENTIFIER_ID 15346 GARD
DX 20130101 MSH
EFFECTIVE_TIME 20020131 SNOMEDCT_US
FX D053537 MSH
GENELOCUS ,1,7,q,1,2,-,q,2,1, OMIM
GENESYMBOL KRT17 OMIM
GENESYMBOL PC2 OMIM
GENESYMBOL PCHC1 OMIM
HAS_GARD_PAGE true GARD
HN 2013 MSH
HPO_COMMENT Steatocystoma multiplex can present with localized, generalized, facial, acral, or suppurative involvement. HPO
IAN DEFAULT ICD10
IDENTIFIER_TYPE_ID 1 GARD
IS_ACTIVE true GARD
IS_RARE true GARD
IS_SPANISH false GARD
MDA 20120703 MSH
MESH_DEFINITION A disorder characterized by multiple, wide spread cutaneous cysts that often become inflamed and rupture. It is caused by the same mutations in the gene coding for KRT-17 that are causative mutations for Pachyonychia congenita, Type 2. Natal teeth involvement is sometimes associated with steatocystoma multiplex. NDFRT
MESH_DUI D062685 NDFRT
MESH_NAME Steatocystoma Multiplex NDFRT
MESH_UI M0528908 NDFRT
MIMTYPE 3 OMIM
MIMTYPEMEANING Phenotype description, molecular basis known. OMIM
MIMTYPEVALUE pound OMIM
MMR 20150618 MSH
MN C16.131.831.350.856.500 MSH
MN C16.320.850.250.856.500 MSH
MN C17.800.529.594.500 MSH
MN C17.800.804.350.856.500 MSH
MN C17.800.827.250.856.500 MSH
NDFRT_KIND DISEASE_KIND NDFRT
NUI N0000185611 NDFRT
ORDER_NO 14282 ICD10CM
PM 2013 MSH
PRIMARY_PATH 10048905$10040834$10040789$10010331$Steatocystoma multiplex$Skin and subcutaneous tissue disorders congenital NEC$Skin and subcutaneous tissue disorders congenital$Congenital, familial and genetic disorders MDR
PRIMARY_SOC 10010331 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/5003/steatocystoma-multiplex/resources/1 GARD
RXAUI 5039934 RXNORM
RXAUI 5039973 RXNORM
RXAUI 5039974 RXNORM
RXAUI 5931664 RXNORM
RXAUI 5931666 RXNORM
RXCUI 1379921 RXNORM
SMQ_TERM_ADDVERSION 10.0 MDR
SMQ_TERM_CAT A MDR
SMQ_TERM_LEVEL 4 MDR
SMQ_TERM_LMVERSION 14.0 MDR
SMQ_TERM_SCOPE 2 MDR
SMQ_TERM_STATUS A MDR
SMQ_TERM_WEIGHT 0 MDR
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS L72.2 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~L72.2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS L72.2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF STEATOCYSTOMA MULTIPLEX WITH NATAL TOOTH SYNDROME CHOOSE K00.6 | 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~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 719306007 | Steatocystoma multiplex with natal tooth syndrome | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~OTHERWISE TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~K00.6 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~L72.2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~ SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~X78S1 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 T734017 MSH
TERMUI T812154 MSH
TERMUI T833787 MSH
TERMUI T842908 MSH
TH GHR (2014) MSH
TH NLM (2010) MSH
TH OMIM (2013) MSH
TH ORD (2010) 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=C0259771

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