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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

Efficacy of vitamin E combined with heparin sodium cream in treatment of scleredema neonatorum.
He J, Li H, Xu M, Zhang Y, Liu T, Li H
Panminerva Med 2021 Jun;63(2):240-241. Epub 2019 Jul 26 doi: 10.23736/S0031-0808.19.03687-5. PMID: 31355597
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
Failure of low-dose methotrexate in the treatment of scleredema diabeticorum in seven cases.
Breuckmann F, Appelhans C, Harati A, Rotterdam S, Altmeyer P, Kreuter A
Dermatology 2005;211(3):299-301. doi: 10.1159/000087031. PMID: 16205082

Recent clinical studies

Etiology

New and emerging conditions of acquired cutaneous mucinoses in adults.
Rongioletti F
J Eur Acad Dermatol Venereol 2022 Jul;36(7):1016-1024. Epub 2022 Feb 17 doi: 10.1111/jdv.17983. PMID: 35124832
Scleroderma mimics.
Nashel J, Steen V
Curr Rheumatol Rep 2012 Feb;14(1):39-46. doi: 10.1007/s11926-011-0220-8. PMID: 22131103
Scleredema adultorum of Buschke treated with radiation.
Bowen AR, Smith L, Zone JJ
Arch Dermatol 2003 Jun;139(6):780-4. doi: 10.1001/archderm.139.6.780. PMID: 12810510
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
Endocrine origins of rheumatic disease. Diagnostic clues to interrelated syndromes.
Lockshin MD
Postgrad Med 2002 Apr;111(4):87-8, 91-2. doi: 10.3810/pgm.2002.04.1156. PMID: 11985136

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 mimics.
Nashel J, Steen V
Curr Rheumatol Rep 2012 Feb;14(1):39-46. doi: 10.1007/s11926-011-0220-8. PMID: 22131103
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
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

Therapy

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
Morphological clues in the diagnosis of sclerodermiform dermatitis.
Fernandez-Flores A, Gatica-Torres M, Ruelas-Villavicencio AL, Saeb-Lima M
Am J Dermatopathol 2014 Jun;36(6):449-64. doi: 10.1097/DAD.0000000000000049. PMID: 24423930
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
Idiopathic scleredema.
Ng E, Rosenstein R, Terushkin V, Meehan S, Pomeranz MK
Dermatol Online J 2016 Dec 15;22(12) PMID: 28329532
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
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
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

Scleredema of Buschke associated with lichen sclerosus: Three cases.
Venturi M, Damevska K, Ferreli C, Pinna AL, Atzori L, Gocev G, Rongioletti F
Indian J Dermatol Venereol Leprol 2020 May-Jun;86(3):272-277. doi: 10.4103/ijdvl.IJDVL_288_17. PMID: 30289118
Scleredema adultorum of Buschke treated with radiation.
Bowen AR, Smith L, Zone JJ
Arch Dermatol 2003 Jun;139(6):780-4. doi: 10.1001/archderm.139.6.780. PMID: 12810510
Scleroedema in a child.
Mitsuhashi Y, Kondo S, Shimizu Y
J Dermatol 1996 Jul;23(7):495-8. doi: 10.1111/j.1346-8138.1996.tb04063.x. PMID: 8772032
Mucin deposits in morphea and systemic scleroderma.
Rongioletti F, Gambini C, Micalizzi C, Pastorino A, Rebora A
Dermatology 1994;189(2):157-8. doi: 10.1159/000246821. PMID: 8075444
Monoclonal gammopathy in scleredema. Observations in three cases.
Kövary PM, Vakilzadeh F, Macher E, Zaun H, Merk H, Goerz G
Arch Dermatol 1981 Sep;117(9):536-9. doi: 10.1001/archderm.1981.01650090018016. PMID: 6794460

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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