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Nodular cutaneous amyloidosis

MedGen UID:
961663
Concept ID:
CN279690
Disease or Syndrome
Synonyms: PLCNA; Primary localized cutaneous nodular amyloidosis
 
Orphanet: ORPHA137810

Definition

Primary localized cutaneous nodular amyloidosis (PLCNA) is the most rare form of primary cutaneous amyloidosis (see this term), a skin disease characterized by the accumulation of amyloid deposits in the dermis, characterized clinically by yellowish waxy crusted nodules and papules on the face, lower extremities, trunk, scalp, and genitalia and histologically by the localized deposition of immunoglobulin-derived amyloid in the papillary dermis and subcutis. PLCNA can be associated with connective tissue disorders such as Sjögren?s syndrome and CREST syndrome (see these terms). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNodular cutaneous amyloidosis

Professional guidelines

PubMed

Weidner T, Illing T, Elsner P
Am J Clin Dermatol 2017 Oct;18(5):629-642. doi: 10.1007/s40257-017-0278-9. PMID: 28342017

Recent clinical studies

Etiology

Konopinski JC, Seyfer SJ, Robbins KL, Hsu S
Dermatol Online J 2013 Apr 15;19(4):10. PMID: 24021370
Borowicz J, Gillespie M, Miller R
Skinmed 2011 Mar-Apr;9(2):96-100; quiz 101. PMID: 21548513
Meijer JM, Schonland SO, Palladini G, Merlini G, Hegenbart U, Ciocca O, Perfetti V, Leijsma MK, Bootsma H, Hazenberg BP
Arthritis Rheum 2008 Jul;58(7):1992-9. doi: 10.1002/art.23617. PMID: 18576343
Criado PR, Silva CS, Vasconcellos C, Valente NY, Maito JB
J Eur Acad Dermatol Venereol 2005 Jul;19(4):481-3. doi: 10.1111/j.1468-3083.2004.01182.x. PMID: 15987299
Kakani RS, Goldstein AE, Meisher I, Hoffman C
J Cutan Med Surg 2001 Mar-Apr;5(2):101-4. Epub 2001 Mar 2 doi: 10.1007/BF02737862. PMID: 11443480

Diagnosis

Jiang Y, Kong Q, Hui Y, Sang H
An Bras Dermatol 2018 Jan-Feb;93(1):111-113. doi: 10.1590/abd1806-4841.20186504. PMID: 29641709Free PMC Article
Konopinski JC, Seyfer SJ, Robbins KL, Hsu S
Dermatol Online J 2013 Apr 15;19(4):10. PMID: 24021370
Borowicz J, Shama L, Miller R
Skinmed 2011 Sep-Oct;9(5):316-8. PMID: 22165048
Borowicz J, Gillespie M, Miller R
Skinmed 2011 Mar-Apr;9(2):96-100; quiz 101. PMID: 21548513
Meijer JM, Schonland SO, Palladini G, Merlini G, Hegenbart U, Ciocca O, Perfetti V, Leijsma MK, Bootsma H, Hazenberg BP
Arthritis Rheum 2008 Jul;58(7):1992-9. doi: 10.1002/art.23617. PMID: 18576343

Therapy

Nandeesh BN, Rajalakshmi T, Shubha B
Indian J Pathol Microbiol 2014 Jan-Mar;57(1):127-9. doi: 10.4103/0377-4929.130920. PMID: 24739851
Borowicz J, Shama L, Miller R
Skinmed 2011 Sep-Oct;9(5):316-8. PMID: 22165048

Prognosis

Trignano E, Ciudad P, Fallico N, Chen HC
J Oral Maxillofac Surg 2012 Aug;70(8):e481-3. Epub 2012 Jun 12 doi: 10.1016/j.joms.2012.03.024. PMID: 22695011
Borowicz J, Shama L, Miller R
Skinmed 2011 Sep-Oct;9(5):316-8. PMID: 22165048
Persoons JH, Sutorius FJ, Koopman RJ, Schaafsma MR, van Doorn GA
Am J Obstet Gynecol 1999 Apr;180(4):1041-4. doi: 10.1016/s0002-9378(99)70684-8. PMID: 10203681

Clinical prediction guides

Nandeesh BN, Rajalakshmi T, Shubha B
Indian J Pathol Microbiol 2014 Jan-Mar;57(1):127-9. doi: 10.4103/0377-4929.130920. PMID: 24739851
Meijer JM, Schonland SO, Palladini G, Merlini G, Hegenbart U, Ciocca O, Perfetti V, Leijsma MK, Bootsma H, Hazenberg BP
Arthritis Rheum 2008 Jul;58(7):1992-9. doi: 10.1002/art.23617. PMID: 18576343
Criado PR, Silva CS, Vasconcellos C, Valente NY, Maito JB
J Eur Acad Dermatol Venereol 2005 Jul;19(4):481-3. doi: 10.1111/j.1468-3083.2004.01182.x. PMID: 15987299
Mochizuki H, Kamakura K, Masaki T, Hirata A, Tokuda T, Yazaki M, Motoyoshi K, Ikeda S
Amyloid 2001 Jun;8(2):105-10. doi: 10.3109/13506120109007352. PMID: 11409031
Badell A, Servitje O, Graells J, Curcó N, Notario J, Peyrí J
Br J Dermatol 1996 Aug;135(2):327-9. PMID: 8881687

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