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Pseudoxanthomatous diffuse cutaneous mastocytosis

MedGen UID:
1842453
•Concept ID:
C5679949
•
Neoplastic Process
Synonyms: Infiltrative small vesicular DCM; infiltrative small vesicular DCM; Infiltrative small vesicular diffuse cutaneous mastocytosis; infiltrative small vesicular diffuse cutaneous mastocytosis; Pseudoxanthomatous DCM; pseudoxanthomatous diffuse cutaneous mastocytosis
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:0017244
Orphanet: ORPHA280794

Definition

Pseudoxanthomatous diffuse cutaneous mastocytosis (PDCM) is a rare form of diffuse cutaneous mastocytosis (DCM; see this term) characterized by yellow-orange infiltrated and xanthogranuloma-like lesions with only limited blistering. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPseudoxanthomatous diffuse cutaneous mastocytosis

Recent clinical studies

Etiology

Diffuse cutaneous mastocytosis: analysis of 10 cases and a brief review of the literature.
Lange M, Niedoszytko M, Nedoszytko B, Łata J, Trzeciak M, Biernat W
J Eur Acad Dermatol Venereol 2012 Dec;26(12):1565-71. Epub 2011 Nov 18 doi: 10.1111/j.1468-3083.2011.04350.x. PMID: 22092511

Diagnosis

Pseudoxanthomatous or xanthelasmoid mastocytosis: Reporting a rare entity.
Sinha P, Sandhu S, Sen A, Sood A
Indian J Dermatol Venereol Leprol 2020 Jul-Aug;86(4):382-385. doi: 10.4103/ijdvl.IJDVL_633_17. PMID: 30785120
Diffuse cutaneous mastocytosis: analysis of 10 cases and a brief review of the literature.
Lange M, Niedoszytko M, Nedoszytko B, Łata J, Trzeciak M, Biernat W
J Eur Acad Dermatol Venereol 2012 Dec;26(12):1565-71. Epub 2011 Nov 18 doi: 10.1111/j.1468-3083.2011.04350.x. PMID: 22092511
Coexistence of two types of clinical lesions in childhood-onset mastocytosis.
Pérez-Pérez L, Allegue F, Caeiro JL, Fabeiro JM, Pérez Rodríguez A, Zulaica A
Indian J Dermatol Venereol Leprol 2011 Mar-Apr;77(2):184-7. doi: 10.4103/0378-6323.77460. PMID: 21393950
Letter: A case of pseudoxanthomatous mastocytosis.
Niemi KM, Karvonen J
Br J Dermatol 1976 Mar;94(3):343-4. doi: 10.1111/j.1365-2133.1976.tb04396.x. PMID: 1252367
Pseudoxanthomatous mastocytosis.
Griffiths WA, Daneshbod K
Br J Dermatol 1975 Jul;93(1):91-5. doi: 10.1111/j.1365-2133.1975.tb06482.x. PMID: 1081401

Therapy

Pseudoxanthomatous mastocytosis.
Srivastava N, Chand S, Singh S
Int J Dermatol 2008 Jan;47(1):50-1. doi: 10.1111/j.1365-4632.2007.03358.x. PMID: 18173602

Prognosis

Diffuse cutaneous mastocytosis: analysis of 10 cases and a brief review of the literature.
Lange M, Niedoszytko M, Nedoszytko B, Łata J, Trzeciak M, Biernat W
J Eur Acad Dermatol Venereol 2012 Dec;26(12):1565-71. Epub 2011 Nov 18 doi: 10.1111/j.1468-3083.2011.04350.x. PMID: 22092511

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