{"Name":"Lymphomatoid papulosis","DiseaseID__c":"GARD:0006944","id":6944,"encodedName":"lymphomatoid-papulosis","IsDeleted":false,"Disease_Name_Full__c":"Lymphomatoid papulosis","Xref_IDs__c":"31047003; 397353001; C0206182; C3721; D017731; MEDGEN:61534; MONDO:0020326; ORPHA:98842","USA_Estimate__c":null,"No_of_Specialist_Tagsa__c":4,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":0,"World_Estimate__c":null,"No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":3,"Disease_Characteristics_Score__c":5,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0020326","Disease_Description__c":"Lymphomatoid papulosis (LyP) is a rare cutaneous condition characterized by chronic, recurrent, and self-regressing papulonodular skin eruptions. It belongs to the spectrum of primary cutaneous CD30+ lymphoproliferative disorders, along with primary cutaneous anaplastic large cell lymphoma (primary C-ALCL; see this term) with which it shares overlapping clinical and histopathologic features.","GARD_Name__c":"Lymphomatoid papulosis","GARD_Synonym__c":"lyp","Curated_Disease_Description_Source__c":"GARD:0006944","Curated_Disease_Description__c":"Lymphomatoid papulosis (LyP) is a non-contagious, chronic skin condition characterized by the eruption of recurring, self-healing bumps (lesions) on the skin. The lesions typically begin small and then become larger, and they may bleed or ulcerate before becoming scaly and crusty. They often develop a red-brown color. Symptoms associated with lesions may include itching and/or pain, which may be debilitating. The frequency of eruptions varies over time and from person to person. Lesions may be very persistent, or they may go away for long periods of time before coming back. They may occur anywhere on the body, but they typically develop on the trunk, arms, and legs, and may develop on the hands, face, and genitalia. They generally go away on their own over a period of weeks to months (usually between 3 and 8 weeks), with or without scarring. LyP is not contagious. The cause of LyP is not known, but there is no evidence that it is hereditary. Stress is often reported to trigger eruptions of lesions. A diagnosis of LyP requires evaluating the symptoms and having a skin biopsy for various types of laboratory tests. While LyP usually is not classified as a cancer (although there has been some debate), it has characteristics of lymphoma under the microscope, and people with LyP have a life-long increased risk of developing lymphoma such as mycosis fungoides, PC-ALCL, or Hodgkin lymphoma. In 5 to 20 percent of people with LyP, the condition is either preceded by lymphoma, associated with lymphoma, or followed by lymphoma. When LyP is diagnosed it is important to rule out these cancers, and for this reason, various blood tests or imaging studies may also be recommended.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":null,"Age_at_Onset_Snippet_Text__c":"at any time in life","SourceID__c":"ORPHA:98842","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0020326","ORPHANET_ID__c":"ORPHA:98842","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Papulosis linfomatoide","Spanish_Description_Source__c":"ORPHA:98842","Spanish_Description__c":"La papulosis linfomatoide (PL) es una enfermedad cutánea rara caracterizada por erupciones cutáneas pápulo-nodulares crónicas, recurrentes y autoinvolutivas. Pertenece al espectro de los procesos linfoproliferativos cutáneos primarios CD30+, junto con el linfoma anaplásico de células grandes, tipo cutáneo primario (LACG-C primario; véase este término), con el que comparte rasgos clínicos e histopatológicos.","Spanish_Disease_Name__c":"papulosis linfomatoide","Spanish_GARD_Synonym__c":"lyp","Category_Linearization__c":"ORPHA:250908","icd10_id__c":null,"mesh_id__c":null,"omim_id__c":null,"snomed_id__c":null,"umls_id__c":null,"GARD_Disease__c":[{"Curated_Disease_Description__c":"Lymphomatoid papulosis (LyP) is a non-contagious, chronic skin condition characterized by the eruption of recurring, self-healing bumps (lesions) on the skin. The lesions typically begin small and then become larger, and they may bleed or ulcerate before becoming scaly and crusty. They often develop a red-brown color. Symptoms associated with lesions may include itching and/or pain, which may be debilitating. The frequency of eruptions varies over time and from person to person. Lesions may be very persistent, or they may go away for long periods of time before coming back. They may occur anywhere on the body, but they typically develop on the trunk, arms, and legs, and may develop on the hands, face, and genitalia. They generally go away on their own over a period of weeks to months (usually between 3 and 8 weeks), with or without scarring. LyP is not contagious. The cause of LyP is not known, but there is no evidence that it is hereditary. Stress is often reported to trigger eruptions of lesions. A diagnosis of LyP requires evaluating the symptoms and having a skin biopsy for various types of laboratory tests. While LyP usually is not classified as a cancer (although there has been some debate), it has characteristics of lymphoma under the microscope, and people with LyP have a life-long increased risk of developing lymphoma such as mycosis fungoides, PC-ALCL, or Hodgkin lymphoma. In 5 to 20 percent of people with LyP, the condition is either preceded by lymphoma, associated with lymphoma, or followed by lymphoma. When LyP is diagnosed it is important to rule out these cancers, and for this reason, various blood tests or imaging studies may also be recommended.","Curated_Disease_Description_Source__c":"GARD:0006944","GARD_Synonym__c":"lyp","Name":"Lymphomatoid papulosis","estimateUsa":""}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"Lymphoma Research Foundation","Website__c":"https://lymphoma.org/"},{"Account_Name__c":"American Cancer Society","Website__c":"https://www.cancer.org"},{"Account_Name__c":"Cutaneous Lymphoma Foundation","Website__c":"https://www.clfoundation.org/"}],"GARD_Disease_Tag__c":[{"Tag_Name__c":"Cancer - Oncologist","Tag_Category__c":"Specialist"},{"Tag_Name__c":"Cancer","Tag_Category__c":"Disease Category","category_description":"Cancer is a disease in which some of the body's cells grow uncontrollably and can spread to other parts of the body.","curated_tag_name":"Cancer"},{"Tag_Name__c":"Dermatology","Tag_Category__c":"Account;Disease Category;Specialist","category_description":"Skin diseases, or integumentary system diseases, affect the skin, hair, nails, sweat glands, or oil glands.","curated_tag_name":"Skin diseases"},{"Tag_Name__c":"Hematology","Tag_Category__c":"Disease Category;Specialist","category_description":"Blood diseases affect the blood or blood-forming organs, including red blood cells, white blood cells, platelets, plasma, and bone marrow.","curated_tag_name":"Blood diseases"},{"Tag_Name__c":"Pediatrics","Tag_Category__c":"Specialist"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"All ages","Provided_By__c":"ORPHA:98842"}],"External_Identifier_Disease__c":[{"URL__c":"https://raresource.nih.gov/diseases/filter/0006944","Source__c":"RareSource"},{"URL__c":"https://www.ncbi.nlm.nih.gov/mesh/C017731","Source__c":"C0206182; MONDO:0020326","Xref__c":"D017731"},{"URL__c":"https://www.orpha.net/en/disease/detail/98842","Source__c":"C0206182; MONDO:0020326; ORPHA:98842","Xref__c":"ORPHA:98842"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=61534","Source__c":"C0206182","Xref__c":"MEDGEN:61534"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=31047003","Source__c":"C0206182; MONDO:0020326","Xref__c":"31047003"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0206182","Source__c":"C0206182","Xref__c":"C0206182"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C3721","Source__c":"C0206182; MONDO:0020326","Xref__c":"C3721"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=397353001","Source__c":"C0206182","Xref__c":"397353001"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0020326","Source__c":"GARD:0006944","Xref__c":"MONDO:0020326"}],"tags":{"Specialist":["Cancer - Oncologist","Dermatology","Hematology","Pediatrics"],"Disease Category":["Cancer","Dermatology","Hematology"],"Account":["Dermatology"]},"synonyms":["lyp"]}