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Scleromyxedema

MedGen UID:
120476
Concept ID:
C0263390
Disease or Syndrome
Synonym: Papular mucinosis
SNOMED CT: Scleromyxedema (402468007); Arndt-Gottron syndrome (402468007); Generalized papular and sclerodermoid lichen myxedematosus (402468007); Generalized lichenoid papular eruption (402468007)
 
Monarch Initiative: MONDO:0015665
Orphanet: ORPHA167635

Definition

A rare lichen myxedematosus characterized by a progressive, generalized, papular, sclerodermoid cutaneous eruption usually occurring in association with monoclonal gammopathy, but in the absence of thyroid disease. Histological hallmark is the triad of dermal mucin deposition, fibroblast proliferation, and fibrosis. Patients present with relatively sudden onset of numerous closely spaced, waxy, firm papules and plaques predominantly involving the head, neck, trunk, and dorsal aspects of the extremities, on the background of thickened, edematous, erythematous skin with sclerodermoid appearance. Systemic involvement with cardiovascular, gastrointestinal, pulmonary, musculoskeletal, renal, or nervous system complications is common. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
Follow this link to review classifications for Scleromyxedema in Orphanet.

Professional guidelines

PubMed

Haber R, Bachour J, El Gemayel M
Int J Dermatol 2020 Oct;59(10):1191-1201. Epub 2020 May 2 doi: 10.1111/ijd.14888. PMID: 32358980
Hoffmann JHO, Enk AH
J Dtsch Dermatol Ges 2017 Dec;15(12):1211-1226. doi: 10.1111/ddg.13389. PMID: 29228499
Marckmann P, Skov L
Radiol Clin North Am 2009 Sep;47(5):833-40, vi. doi: 10.1016/j.rcl.2009.05.004. PMID: 19744598

Recent clinical studies

Etiology

Machado RA, Oliveira LQ, Martelli-Júnior H, Pires FR, Carvas JB, Rogerio VE, Rabelo VD, Coletta RD
Med Oral Patol Oral Cir Bucal 2023 May 1;28(3):e278-e284. doi: 10.4317/medoral.25713. PMID: 36565218Free PMC Article
Hoffmann JHO, Enk AH
J Dtsch Dermatol Ges 2020 Dec;18(12):1449-1467. doi: 10.1111/ddg.14319. PMID: 33373143
Haber R, Bachour J, El Gemayel M
Int J Dermatol 2020 Oct;59(10):1191-1201. Epub 2020 May 2 doi: 10.1111/ijd.14888. PMID: 32358980
Wick MR, Patterson JW
Semin Diagn Pathol 2019 Jul;36(4):211-228. Epub 2019 Jan 31 doi: 10.1053/j.semdp.2019.01.001. PMID: 30736994
Ferreli C, Gasparini G, Parodi A, Cozzani E, Rongioletti F, Atzori L
Clin Rev Allergy Immunol 2017 Dec;53(3):306-336. doi: 10.1007/s12016-017-8625-4. PMID: 28712039

Diagnosis

Chatterjee S, Fernandez AP
N Engl J Med 2023 Nov 23;389(21):1992. doi: 10.1056/NEJMicm2302207. PMID: 37991858
Liu CH, Tsai YY, Tien YC
Rheumatology (Oxford) 2022 Jul 6;61(7):e201-e200. doi: 10.1093/rheumatology/keab718. PMID: 34554227
Hoffmann JHO, Enk AH
J Dtsch Dermatol Ges 2020 Dec;18(12):1449-1467. doi: 10.1111/ddg.14319. PMID: 33373143
Rongioletti F, Ferreli C, Atzori L, Bottoni U, Soda G
G Ital Dermatol Venereol 2018 Apr;153(2):208-215. Epub 2018 Jan 24 doi: 10.23736/S0392-0488.18.05922-9. PMID: 29368844
Nashel J, Steen V
Curr Rheumatol Rep 2012 Feb;14(1):39-46. doi: 10.1007/s11926-011-0220-8. PMID: 22131103

Therapy

Machado RA, Oliveira LQ, Martelli-Júnior H, Pires FR, Carvas JB, Rogerio VE, Rabelo VD, Coletta RD
Med Oral Patol Oral Cir Bucal 2023 May 1;28(3):e278-e284. doi: 10.4317/medoral.25713. PMID: 36565218Free PMC Article
Yang Y, Chen H, Zhang W, Sun JF
Indian J Dermatol Venereol Leprol 2016 Nov-Dec;82(6):738-739. doi: 10.4103/0378-6323.174420. PMID: 26858056
Qureshi F, Dharmasena A, Leatherbarrow B
Ophthalmic Plast Reconstr Surg 2015 Sep-Oct;31(5):e119-20. doi: 10.1097/IOP.0000000000000144. PMID: 24807804
Heymann WR
J Am Acad Dermatol 2007 Nov;57(5):890-1. doi: 10.1016/j.jaad.2007.04.033. PMID: 17939940
Bata-Csorgo Z, Husz S, Foldes M, Korom I, Molnar K, Morvay M, Dobozy A
J Am Acad Dermatol 1999 Aug;41(2 Pt 2):343-6. doi: 10.1016/s0190-9622(99)70383-x. PMID: 10426928

Prognosis

Hoffmann JHO, Enk AH
J Dtsch Dermatol Ges 2020 Dec;18(12):1449-1467. doi: 10.1111/ddg.14319. PMID: 33373143
Hoffmann JHO, Enk AH
Front Immunol 2019;10:1090. Epub 2019 Jun 11 doi: 10.3389/fimmu.2019.01090. PMID: 31244821Free PMC Article
Ferreli C, Gasparini G, Parodi A, Cozzani E, Rongioletti F, Atzori L
Clin Rev Allergy Immunol 2017 Dec;53(3):306-336. doi: 10.1007/s12016-017-8625-4. PMID: 28712039
Pomann JJ, Rudner EJ
Int J Dermatol 2003 Jan;42(1):31-5. doi: 10.1046/j.1365-4362.2003.01565.x. PMID: 12581140
Jackson EM, English JC 3rd
Dermatol Clin 2002 Jul;20(3):493-501. doi: 10.1016/s0733-8635(02)00017-7. PMID: 12170882

Clinical prediction guides

Mecoli CA, Talbot CC Jr, Fava A, Cheadle C, Boin F, Wigley FM, Hummers LK
Arthritis Care Res (Hoboken) 2020 Jun;72(6):761-767. Epub 2020 May 14 doi: 10.1002/acr.23908. PMID: 31008568Free PMC Article
Soulages A, Tang HM, Pham-Ledard A, Négrier-Leibreich ML, Cosnard A, Duval F, Solé G, Carla L, Le Masson G, Mathis S
J Neurol 2019 Aug;266(8):2051-2059. Epub 2019 May 21 doi: 10.1007/s00415-019-09379-w. PMID: 31115676
Hermans C, Goldscheider I, Ruzicka T, Rongioletti F
J Eur Acad Dermatol Venereol 2016 Dec;30(12):2048-2051. Epub 2016 Jul 16 doi: 10.1111/jdv.13833. PMID: 27422288
Abarzúa AA, Giesen LF, Sandoval MO, González SB
Int J Dermatol 2014 Aug;53(8):971-4. Epub 2014 Feb 14 doi: 10.1111/ijd.12124. PMID: 24527753
Bardin T
Curr Opin Rheumatol 2003 Jan;15(1):48-54. doi: 10.1097/00002281-200301000-00009. PMID: 12496510

Recent systematic reviews

Machado RA, Oliveira LQ, Martelli-Júnior H, Pires FR, Carvas JB, Rogerio VE, Rabelo VD, Coletta RD
Med Oral Patol Oral Cir Bucal 2023 May 1;28(3):e278-e284. doi: 10.4317/medoral.25713. PMID: 36565218Free PMC Article
Haber R, Bachour J, El Gemayel M
Int J Dermatol 2020 Oct;59(10):1191-1201. Epub 2020 May 2 doi: 10.1111/ijd.14888. PMID: 32358980

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