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Cutaneous nodular amyloidosis(NLCA)

MedGen UID:
900275
•Concept ID:
C4274331
•
Disease or Syndrome
Synonyms: Amyloidosis cutis nodularis atrophicans; Amyloidosis nodular localized cutaneous; NLCA; Primary localized cutaneous nodular amyloidosis
SNOMED CT: Primary localized cutaneous nodular amyloidosis (716704007); PLCNA - Primary localized cutaneous nodular amyloidosis (716704007); Nodular amyloidosis (716704007)
 
HPO: HP:0032348
Monarch Initiative: MONDO:0015302

Definition

A type of cutaneous amyloidosis that is characterized clinically by waxy, purpuric plaques and nodules and histologically by amyloid deposits in the dermis and subcutaneous tissue. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCutaneous nodular amyloidosis

Professional guidelines

PubMed

Primary Localized Cutaneous Amyloidosis: A Systematic Treatment Review.
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

Nodular amyloidosis in a patient with systemic scleroderma.
Marano AL, Selim MA, Cardones AR, Burton CS
Dermatol Online J 2018 Sep 15;24(9) PMID: 30677833
Cutaneous features and diagnosis of primary Sjögren syndrome: An update and review.
Jhorar P, Torre K, Lu J
J Am Acad Dermatol 2018 Oct;79(4):736-745. Epub 2018 May 19 doi: 10.1016/j.jaad.2018.05.021. PMID: 29787840
Primary Localized Cutaneous Amyloidosis: A Systematic Treatment Review.
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
Nodular amyloidosis derived from keratinocytes: an unusual type of primary localized cutaneous nodular amyloidosis.
Cornejo KM, Lagana FJ, Deng A
Am J Dermatopathol 2015 Nov;37(11):e129-33. doi: 10.1097/DAD.0000000000000307. PMID: 26485243

Diagnosis

Localized Cutaneous Nodular Amyloidosis in a Patient with Sjögren's Syndrome.
Llamas-Molina JM, Velasco-Amador JP, De La Torre-Gomar FJ, Carrero-Castaño A, Ruiz-Villaverde R
Int J Mol Sci 2023 May 28;24(11) doi: 10.3390/ijms24119409. PMID: 37298361Free PMC Article
Localized Cutaneous Nodular Amyloidosis: A Specific Cutaneous Manifestation of Sjögren's Syndrome.
Llamas-Molina JM, Velasco-Amador JP, De la Torre-Gomar FJ, Carrero-Castaño A, Ruiz-Villaverde R
Int J Mol Sci 2023 Apr 17;24(8) doi: 10.3390/ijms24087378. PMID: 37108553Free PMC Article
Localized Cutaneous Nodular Amyloidosis and Sjögren's Syndrome.
Ogawa A, Takahashi Y, Nasu R, Aozasa N
Intern Med 2021 Aug 1;60(15):2513-2514. Epub 2021 Feb 15 doi: 10.2169/internalmedicine.6839-20. PMID: 33583907Free PMC Article
Cutaneous features and diagnosis of primary Sjögren syndrome: An update and review.
Jhorar P, Torre K, Lu J
J Am Acad Dermatol 2018 Oct;79(4):736-745. Epub 2018 May 19 doi: 10.1016/j.jaad.2018.05.021. PMID: 29787840
Primary localized cutaneous nodular amyloidosis and CREST syndrome: a case report and review of the literature.
Summers EM, Kendrick CG
Cutis 2008 Jul;82(1):55-9. PMID: 18712025

Therapy

Cutaneous features and diagnosis of primary Sjögren syndrome: An update and review.
Jhorar P, Torre K, Lu J
J Am Acad Dermatol 2018 Oct;79(4):736-745. Epub 2018 May 19 doi: 10.1016/j.jaad.2018.05.021. PMID: 29787840
Primary Localized Cutaneous Amyloidosis: A Systematic Treatment Review.
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
Primary localized cutaneous nodular amyloidosis of the feet: a case report and review of the literature.
Ritchie SA, Beachkofsky T, Schreml S, Gaspari A, Hivnor CM
Cutis 2014 Feb;93(2):89-94. PMID: 24605345
Primary localized cutaneous nodular amyloidosis successfully treated with cyclophosphamide.
Tong PL, Walker WA, Glancy RJ, Cooney JP, Gebauer K
Australas J Dermatol 2013 Feb;54(1):e12-5. Epub 2011 May 31 doi: 10.1111/j.1440-0960.2011.00770.x. PMID: 23373889
Primary sjögren syndrome manifested as localized cutaneous nodular amyloidosis.
Wey SJ, Chen YM, Lai PJ, Chen DY
J Clin Rheumatol 2011 Oct;17(7):368-70. doi: 10.1097/RHU.0b013e31823209ba. PMID: 21946462

Prognosis

Cutaneous features and diagnosis of primary Sjögren syndrome: An update and review.
Jhorar P, Torre K, Lu J
J Am Acad Dermatol 2018 Oct;79(4):736-745. Epub 2018 May 19 doi: 10.1016/j.jaad.2018.05.021. PMID: 29787840
Dystrophic Calcification in a Patient with Primary Localized Cutaneous Nodular Amyloidosis: An Uncommon Ultrasound Finding.
Planas-Ciudad S, Roé Crespo E, Mir-Bonafé JF, Muñoz-Garza FZ, Puig Sanz L
Acta Derm Venereol 2018 Jan 12;98(1):144-145. doi: 10.2340/00015555-2790. PMID: 28902953

Clinical prediction guides

Dystrophic Calcification in a Patient with Primary Localized Cutaneous Nodular Amyloidosis: An Uncommon Ultrasound Finding.
Planas-Ciudad S, Roé Crespo E, Mir-Bonafé JF, Muñoz-Garza FZ, Puig Sanz L
Acta Derm Venereol 2018 Jan 12;98(1):144-145. doi: 10.2340/00015555-2790. PMID: 28902953
Effective treatment of nodular amyloidosis with carbon dioxide laser.
Lesiak A, Rakowski A, Brzezinska A, Rogowski-Tylman M, Kolano P, Sysa-Jedrzejowska A, Narbutt J
J Cutan Med Surg 2012 Sep-Oct;16(5):372-4. doi: 10.1177/120347541201600519. PMID: 22971317

Recent systematic reviews

Primary Localized Cutaneous Amyloidosis: A Systematic Treatment Review.
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

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