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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
Nofal A, Amer H, Alakad R, Nofal E, Desouky FE, Yosef A, Albalat W, Gharib K, Mostafa I, Fathy S, Waked M, Ragheb E, Gammaz H
Int J Dermatol 2017 Mar;56(3):284-290. Epub 2016 Sep 25 doi: 10.1111/ijd.13437. PMID: 27667657

Recent clinical studies

Etiology

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
Nashel J, Steen V
Curr Rheumatol Rep 2012 Feb;14(1):39-46. doi: 10.1007/s11926-011-0220-8. PMID: 22131103
Foti R, Leonardi R, Rondinone R, Di Gangi M, Leonetti C, Canova M, Doria A
Autoimmun Rev 2008 Feb;7(4):331-9. Epub 2008 Jan 11 doi: 10.1016/j.autrev.2007.12.004. PMID: 18295739
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

Diagnosis

Chatterjee S, Fernandez AP
N Engl J Med 2023 Nov 23;389(21):1992. doi: 10.1056/NEJMicm2302207. PMID: 37991858
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
Liu CH, Tsai YY, Tien YC
Rheumatology (Oxford) 2022 Jul 6;61(7):e201-e200. doi: 10.1093/rheumatology/keab718. PMID: 34554227
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
Nashel J, Steen V
Curr Rheumatol Rep 2012 Feb;14(1):39-46. doi: 10.1007/s11926-011-0220-8. PMID: 22131103

Therapy

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
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
Bogner RR, Wetter DA, Dingli D
Intern Med 2014;53(21):2561-2. Epub 2014 Nov 1 doi: 10.2169/internalmedicine.53.3063. PMID: 25366027
Chen M, Doherty SD, Hsu S
Dermatol Clin 2010 Jul;28(3):577-86. doi: 10.1016/j.det.2010.03.003. PMID: 20510766
Heymann WR
J Am Acad Dermatol 2007 Nov;57(5):890-1. doi: 10.1016/j.jaad.2007.04.033. PMID: 17939940

Prognosis

Rongioletti F, Ferreli C, Atzori L
Clin Dermatol 2021 Mar-Apr;39(2):211-214. Epub 2020 Oct 16 doi: 10.1016/j.clindermatol.2020.10.001. PMID: 34272012
Cárdenas-Gonzalez RE, Ruelas MEH, Candiani JO
An Bras Dermatol 2019;94(4):462-469. Epub 2019 Oct 17 doi: 10.1590/abd1806-4841.20198478. PMID: 31644623Free PMC Article
Foti R, Leonardi R, Rondinone R, Di Gangi M, Leonetti C, Canova M, Doria A
Autoimmun Rev 2008 Feb;7(4):331-9. Epub 2008 Jan 11 doi: 10.1016/j.autrev.2007.12.004. PMID: 18295739
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

Chen J, Zhang X, Li Q, Liu M, Fu Z
Clin Nucl Med 2023 Sep 1;48(9):806-808. Epub 2023 May 23 doi: 10.1097/RLU.0000000000004712. PMID: 37220249
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
Wang P, Yang H, Ran Y
Arch Dermatol 2011 May;147(5):599-602. doi: 10.1001/archdermatol.2011.110. PMID: 21576580
Ting WW, Stone MS, Madison KC, Kurtz K
Arch Dermatol 2003 Jul;139(7):903-6. doi: 10.1001/archderm.139.7.903. PMID: 12873886

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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