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Scleredema

MedGen UID:
11346
•Concept ID:
C0036413
•
Disease or Syndrome
Synonyms: Buschke's scleredema; Scleredema adultorum; Scleredema adultorum of Buschke; Scleredema diabeticorum; Scleredema diabeticorum of Buschke
SNOMED CT: Scleredema (95323007); Buschke's scleredema (95323007); Scleredema adultorum of Buschke (95323007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0006606
Orphanet: ORPHA352763

Definition

Hard non pitting edema and induration of the skin; a finding associated with Buschke''s disease. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVScleredema

Professional guidelines

PubMed

Intravenous immunoglobulin treatment: Where do dermatologists stand?
Emre S
Dermatol Ther 2019 May;32(3):e12854. Epub 2019 Mar 4 doi: 10.1111/dth.12854. PMID: 30756448
European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis.
Knobler R, Moinzadeh P, Hunzelmann N, Kreuter A, Cozzio A, Mouthon L, Cutolo M, Rongioletti F, Denton CP, Rudnicka L, Frasin LA, Smith V, Gabrielli A, Aberer E, Bagot M, Bali G, Bouaziz J, Braae Olesen A, Foeldvari I, Frances C, Jalili A, Just U, Kähäri V, Kárpáti S, Kofoed K, Krasowska D, Olszewska M, Orteu C, Panelius J, Parodi A, Petit A, Quaglino P, Ranki A, Sanchez Schmidt JM, Seneschal J, Skrok A, Sticherling M, Sunderkötter C, Taieb A, Tanew A, Wolf P, Worm M, Wutte NJ, Krieg T
J Eur Acad Dermatol Venereol 2017 Oct;31(10):1581-1594. Epub 2017 Aug 8 doi: 10.1111/jdv.14466. PMID: 28786499
Sclerema neonatorum: a review of nomenclature, clinical presentation, histological features, differential diagnoses and management.
Zeb A, Darmstadt GL
J Perinatol 2008 Jul;28(7):453-60. Epub 2008 Mar 27 doi: 10.1038/jp.2008.33. PMID: 18368059

Recent clinical studies

Etiology

Youth-Onset Type 2 Diabetes Manifestations in other Specialties: Its Many Disguises.
Weghuber D, Barrientos-Pérez M, Kovarenko M
Ann Nutr Metab 2019;74(4):339-347. Epub 2019 May 16 doi: 10.1159/000500234. PMID: 31096219
Treatment of Scleroedema Adultorum Buschke: A Systematic Review.
Miguel D, Schliemann S, Elsner P
Acta Derm Venereol 2018 Mar 13;98(3):305-309. doi: 10.2340/00015555-2846. PMID: 29136263
Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review.
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
Scleroderma mimics.
Nashel J, Steen V
Curr Rheumatol Rep 2012 Feb;14(1):39-46. doi: 10.1007/s11926-011-0220-8. PMID: 22131103
Diffuse cutaneous mucinoses.
Jackson EM, English JC 3rd
Dermatol Clin 2002 Jul;20(3):493-501. doi: 10.1016/s0733-8635(02)00017-7. PMID: 12170882

Diagnosis

Cutaneous Manifestations of Diabetes.
Hines A, Alavi A, Davis MDP
Med Clin North Am 2021 Jul;105(4):681-697. doi: 10.1016/j.mcna.2021.04.008. PMID: 34059245
Cutaneous Manifestations of Diabetes Mellitus and Prediabetes.
Sanches MM, Roda Â, Pimenta R, Filipe PL, Freitas JP
Acta Med Port 2019 Jun 28;32(6):459-465. doi: 10.20344/amp.10738. PMID: 31292028
Scleroderma with an update about clinico-pathological correlation.
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
Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review.
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
Cutaneous Manifestations of Diabetes Mellitus: A Review.
Lima AL, Illing T, Schliemann S, Elsner P
Am J Clin Dermatol 2017 Aug;18(4):541-553. doi: 10.1007/s40257-017-0275-z. PMID: 28374407

Therapy

Treatment of Scleroedema Adultorum Buschke: A Systematic Review.
Miguel D, Schliemann S, Elsner P
Acta Derm Venereol 2018 Mar 13;98(3):305-309. doi: 10.2340/00015555-2846. PMID: 29136263
Cutaneous Manifestations of Diabetes Mellitus: A Review.
Lima AL, Illing T, Schliemann S, Elsner P
Am J Clin Dermatol 2017 Aug;18(4):541-553. doi: 10.1007/s40257-017-0275-z. PMID: 28374407
Diabetes mellitus.
Ahmed I, Goldstein B
Clin Dermatol 2006 Jul-Aug;24(4):237-46. doi: 10.1016/j.clindermatol.2006.04.009. PMID: 16828404
Scleroderma.
Schmults CA
Dermatol Online J 2003 Oct;9(4):11. PMID: 14594584
Skin and diabetes.
Sibbald RG, Landolt SJ, Toth D
Endocrinol Metab Clin North Am 1996 Jun;25(2):463-72. doi: 10.1016/s0889-8529(05)70334-0. PMID: 8799710

Prognosis

Cutaneous Manifestations of Diabetes Mellitus and Prediabetes.
Sanches MM, Roda Â, Pimenta R, Filipe PL, Freitas JP
Acta Med Port 2019 Jun 28;32(6):459-465. doi: 10.20344/amp.10738. PMID: 31292028
Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review.
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
Scleroderma-like fibrosing disorders.
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
Diabetes mellitus.
Ahmed I, Goldstein B
Clin Dermatol 2006 Jul-Aug;24(4):237-46. doi: 10.1016/j.clindermatol.2006.04.009. PMID: 16828404
Skin and diabetes.
Sibbald RG, Landolt SJ, Toth D
Endocrinol Metab Clin North Am 1996 Jun;25(2):463-72. doi: 10.1016/s0889-8529(05)70334-0. PMID: 8799710

Clinical prediction guides

Screening for the presence of scleroedema adultorum of Buschke in patients with diabetes mellitus: newly diagnosed patients had a high prevalence of dyslipidaemia.
Csonka V, Bódis B, Kovács D, Farkas N, Kálmán E, Czirják L, Varjú C
Lipids Health Dis 2021 May 5;20(1):47. doi: 10.1186/s12944-021-01473-1. PMID: 33952255Free PMC Article
Clinical correlates of human leucocyte antigen (HLA)-G in systemic sclerosis.
Favoino E, Favia IE, Vettori S, Vicenti C, Prete M, Valentini G, Perosa F
Clin Exp Immunol 2015 Jul;181(1):100-9. Epub 2015 May 10 doi: 10.1111/cei.12633. PMID: 25847615Free PMC Article
Effects of low-density lipoprotein apheresis on coronary and carotid atherosclerosis and diabetic scleredema in patients with severe hypercholesterolemia.
Koga N
Ther Apher 2001 Aug;5(4):244-51. doi: 10.1046/j.1526-0968.2001.00341.x. PMID: 11724508
A clinical observation of scleredema adultorum and its relationship to diabetes.
Rho YW, Suhr KB, Lee JH, Park JK
J Dermatol 1998 Feb;25(2):103-7. doi: 10.1111/j.1346-8138.1998.tb02358.x. PMID: 9563277
Diabetic scleredema.
Toyota T, Umezu M, Oikawa N, Sanoyama R, Suzuki S, Suzuki H, Nakajima Y, Goto Y
Tohoku J Exp Med 1983 Dec;141(4):457-61. doi: 10.1620/tjem.141.457. PMID: 6670101

Recent systematic reviews

Treatment of Scleroedema Adultorum Buschke: A Systematic Review.
Miguel D, Schliemann S, Elsner P
Acta Derm Venereol 2018 Mar 13;98(3):305-309. doi: 10.2340/00015555-2846. PMID: 29136263
Ultraviolet A phototherapy for sclerotic skin diseases: a systematic review.
Kroft EB, Berkhof NJ, van de Kerkhof PC, Gerritsen RM, de Jong EM
J Am Acad Dermatol 2008 Dec;59(6):1017-30. Epub 2008 Oct 2 doi: 10.1016/j.jaad.2008.07.042. PMID: 18835066

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