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Primary cutaneous plasmacytosis

MedGen UID:
1672491
Concept ID:
C4736227
Disease or Syndrome
Synonym: primary cutaneous plasmacytosis
SNOMED CT: Primary cutaneous plasmacytosis (1228842003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0018679
Orphanet: ORPHA451602

Definition

A rare acquired skin disease characterized by benign proliferation of mature plasma cells with a typical triad of cutaneous lesions, polyclonal hypergammaglobulinemia and superficial lymphadenopathy without an apparent underlying cause. The skin lesions consist of multiple round-to-oval, red-to-dark-brown macules, papules and plaques most commonly found on the trunk but also the face, neck, and axilla. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary cutaneous plasmacytosis

Recent clinical studies

Etiology

Aricò M, Bongiorno MR
J Eur Acad Dermatol Venereol 2002 Mar;16(2):164-7. doi: 10.1046/j.1468-3083.2002.00411.x. PMID: 12046825

Diagnosis

Tiao J, Shin G, Al Janahi S, Chung KY, Kim DY, Mun JH, Lee JB, Chung HJ
Clin Dermatol 2021 Sep-Oct;39(5):879-886. Epub 2021 May 18 doi: 10.1016/j.clindermatol.2021.05.023. PMID: 34785016
Costin A, Alves J, Cerejeira D
Acta Dermatovenerol Alp Pannonica Adriat 2019 Dec;28(4):177-178. PMID: 31855272
Dhar S, Liani L, Patole K, Dhar S
Indian J Dermatol Venereol Leprol 2017 Nov-Dec;83(6):673-676. doi: 10.4103/ijdvl.IJDVL_874_16. PMID: 28799534
Ahn JJ, Yang YS, Shin MK, Lee SW, Kim NI
J Dermatol 2011 Apr;38(4):364-7. doi: 10.1111/j.1346-8138.2010.01111.x. PMID: 21426385
Aricò M, Bongiorno MR
J Eur Acad Dermatol Venereol 2002 Mar;16(2):164-7. doi: 10.1046/j.1468-3083.2002.00411.x. PMID: 12046825

Therapy

On HR, Lee SE, Kim YC, Kim SC
Pediatr Dermatol 2014 Nov-Dec;31(6):e120-1. doi: 10.1111/pde.12449. PMID: 25424220

Prognosis

António AM, Alves JV, Coelho R, Bártolo E
An Bras Dermatol 2017 May-Jun;92(3):410-412. doi: 10.1590/abd1806-4841.20175355. PMID: 29186262Free PMC Article
Gilliam AC, Mullen RH, Oviedo G, Bhatnagar R, Smith MK, Patton DF, Rodriguez-Soto J, Mostow E
Arch Dermatol 2009 Mar;145(3):299-302. doi: 10.1001/archdermatol.2008.596. PMID: 19289761Free PMC Article
Aricò M, Bongiorno MR
J Eur Acad Dermatol Venereol 2002 Mar;16(2):164-7. doi: 10.1046/j.1468-3083.2002.00411.x. PMID: 12046825
Uhara H, Saida T, Ikegawa S, Yamazaki Y, Mikoshiba H, Nijoh S, Kitano K, Koh CS
Dermatology 1994;189(3):251-5. doi: 10.1159/000246848. PMID: 7949477

Clinical prediction guides

Gilliam AC, Mullen RH, Oviedo G, Bhatnagar R, Smith MK, Patton DF, Rodriguez-Soto J, Mostow E
Arch Dermatol 2009 Mar;145(3):299-302. doi: 10.1001/archdermatol.2008.596. PMID: 19289761Free PMC Article

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