{"orpha":33111,"name":"Granulomatous slack skin","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare variant of mycosis fungoides (MF) characterized by the presence of circumscribed areas of pendulous lax skin.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-33111/","orphanet_url":"https://www.orpha.net/en/disease/detail/33111","names":{"fr":{"name":"Chalazodermie granulomateuse","synonyms":["Dermatochalasie granulomateuse"],"definition":"Variante rare du mycosis fongoïde (MF) caractérisée par la présence de zones circonscrites de peau flasque et pendante."},"de":{"name":"Haut, granulomatöse schlaffe","synonyms":["Kutanes elastolytisches T-Zell-Lymphom"],"definition":"Eine seltene Variante der Mycosis fungoides (MF), die durch das Vorhandensein umschriebener Bereiche mit schlaffer, hängender Haut gekennzeichnet ist."},"es":{"name":"Piel laxa granulomatosa","synonyms":["Granuloma chalazodérmico","Dermatocalasia granulomatosa"],"definition":"Es una variante poco frecuente de micosis fungoide (MF) caracterizada por la presencia de áreas circunscritas de piel laxa y péndula."},"it":{"name":"Granulomatosi con cutis laxa"},"nl":{"name":"Granulomateuze slappe huid","definition":"Een zeldzame variant van mycosis fungoides (MF), gekenmerkt door de aanwezigheid van begrensde gebieden van penduleuze (hangende) en slappe huid."},"pt":{"name":"Pele laxa granulomatosa","definition":"A pele laxa granulomatosa (GSS) é uma variante de micose fungóide (MF; ver este termo), uma forma de linfoma cutâneo de células T, e é caracterizado pela presença de áreas circunscritas de pele laxa e pendular. Até ao momento foram descritos na literatura menos de 50 casos."},"pl":{"name":"Ziarniniakowe zwiotczenie skóry"},"cs":{"name":"Granulomatózní syndrom povislé kůže"},"uk":{"name":"Гранулематозна вяла шкіра","definition":"Granulomatous slack skin (GSS) is a variant of mycosis fungoides (MF; see this term), a form of cutaneous T-cell lymphoma, and is characterized by the presence of circumscribed areas of pendulous lax skin."},"zh":{"name":"肉芽肿性皮肤松弛症"},"tr":{"name":"Granülomatöz gevşek deri"}},"xrefs":[{"source":"ICD-10","code":"C84.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B01","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1557643847"},{"source":"ICD-11","code":"XH0EH1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"858797331"},{"source":"MONDO","code":"0018031","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0376407","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10986","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":178566,"name":"Mycosis fungoides and variants","classifications":[187,194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":50.0,"area":"Worldwide","status":"Validated","source":"14726855[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-08-12","source":"3496402[PMID]","terms":[{"hpo":"HP:0000973","label":"Cutis laxa","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012189","label":"Hodgkin lymphoma","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002665","label":"Lymphoma","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001582","label":"Redundant skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030053","label":"Stiff skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002733","label":"Abnormality of the lymph nodes","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001919","label":"Acute kidney injury","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003072","label":"Hypercalcemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000121","label":"Nephrocalcinosis","frequency":"Occasional (29-5%)","criteria":null}]}}