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Scleromyxedema

MedGen UID:
120476
Concept ID:
C0263390
Disease or Syndrome
Synonym: Papular mucinosis
SNOMED CT: Scleromyxedema (402468007); Lichen myxedematosus (111197009); Arndt-Gottron syndrome (402468007); Papular mucinosis (111197009); Lichenoid myxedema (111197009); 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
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
Nashel J, Steen V
Curr Rheumatol Rep 2012 Feb;14(1):39-46. doi: 10.1007/s11926-011-0220-8. PMID: 22131103

Diagnosis

Claveau JS, Wetter DA, Kumar S
Blood Cancer J 2022 Apr 11;12(4):58. doi: 10.1038/s41408-022-00661-1. PMID: 35411042Free PMC Article
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
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
Hummers LK
Curr Opin Rheumatol 2014 Nov;26(6):658-62. doi: 10.1097/BOR.0000000000000118. PMID: 25215418

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
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
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
Qureshi F, Dharmasena A, Leatherbarrow B
Ophthalmic Plast Reconstr Surg 2015 Sep-Oct;31(5):e119-20. doi: 10.1097/IOP.0000000000000144. PMID: 24807804
Hummers LK
Curr Opin Rheumatol 2014 Nov;26(6):658-62. doi: 10.1097/BOR.0000000000000118. PMID: 25215418

Prognosis

Hoffmann JHO, Enk AH
J Dtsch Dermatol Ges 2020 Dec;18(12):1449-1467. doi: 10.1111/ddg.14319. PMID: 33373143
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
Boin F, Hummers LK
Rheum Dis Clin North Am 2008 Feb;34(1):199-220; ix. doi: 10.1016/j.rdc.2007.11.001. PMID: 18329541Free PMC Article
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

Geisler A, Hosseinipour M, Vyas NS, Phelps R, Gropper C, Hoffman C
J Drugs Dermatol 2020 Mar 1;19(3):320-322. doi: 10.36849/JDD.2020.4864. PMID: 32550685
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
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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