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Coup de sabre scleroderma

MedGen UID:
581116
•Concept ID:
C0392441
•
Disease or Syndrome
Synonyms: Frontal Linear Scleroderma; Morphea en coup de sabre; Scleroderma en Coup de Sabre
SNOMED CT: Morphea en coup de sabre (51156002); Coup de sabre scleroderma (51156002)

Definition

A type of linear scleroderma characterized by a linear, colorless, atrophied band across the forehead or scalp. This disorder can affect the tissues under or near the lesion including brain, bone and eyes. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCoup de sabre scleroderma

Professional guidelines

PubMed

S2k guideline: Diagnosis and therapy of localized scleroderma.
Kreuter A, Moinzadeh P, Kinberger M, Horneff G, Worm M, Werner RN, Hammacher A, Krieg T, Wenzel J, Oeschger M, Weibel L, Müllegger R, Hunzelmann N
J Dtsch Dermatol Ges 2024 Apr;22(4):605-620. Epub 2024 Mar 1 doi: 10.1111/ddg.15328. PMID: 38426689
Immunologic underpinnings and treatment of morphea.
LaChance AH, Goldman N, Kassamali B, Vleugels RA
Expert Rev Clin Immunol 2022 May;18(5):461-483. Epub 2022 May 4 doi: 10.1080/1744666X.2022.2063841. PMID: 35403538
Update on the classification and treatment of localized scleroderma.
Bielsa Marsol I
Actas Dermosifiliogr 2013 Oct;104(8):654-66. Epub 2013 Aug 13 doi: 10.1016/j.adengl.2012.10.012. PMID: 23948159

Recent clinical studies

Etiology

En coup de sabre scleroderma and Parry-Romberg syndrome in adolescents: surgical options and patient-related outcomes.
Palmero ML, Uziel Y, Laxer RM, Forrest CR, Pope E
J Rheumatol 2010 Oct;37(10):2174-9. Epub 2010 Sep 15 doi: 10.3899/jrheum.100062. PMID: 20843909

Diagnosis

A case of multiple cerebral cavernomas associated with en coup de sabre scleroderma.
Alwash N, Dinani N, Felton J
Pediatr Dermatol 2020 Jan;37(1):237-238. Epub 2019 Nov 22 doi: 10.1111/pde.14052. PMID: 31755138
A review of Parry-Romberg syndrome.
El-Kehdy J, Abbas O, Rubeiz N
J Am Acad Dermatol 2012 Oct;67(4):769-84. Epub 2012 Mar 7 doi: 10.1016/j.jaad.2012.01.019. PMID: 22405645
Scleroderma linearis: hemiatrophia faciei progressiva (Parry-Romberg syndrom) without any changes in CNS and linear scleroderma "en coup de sabre" with CNS tumor.
Bergler-Czop B, Lis-Swiety A, Brzezińska-Wcisło L
BMC Neurol 2009 Jul 27;9:39. doi: 10.1186/1471-2377-9-39. PMID: 19635150Free PMC Article

Clinical prediction guides

En coup de sabre scleroderma and Parry-Romberg syndrome in adolescents: surgical options and patient-related outcomes.
Palmero ML, Uziel Y, Laxer RM, Forrest CR, Pope E
J Rheumatol 2010 Oct;37(10):2174-9. Epub 2010 Sep 15 doi: 10.3899/jrheum.100062. PMID: 20843909
Scleroderma linearis: hemiatrophia faciei progressiva (Parry-Romberg syndrom) without any changes in CNS and linear scleroderma "en coup de sabre" with CNS tumor.
Bergler-Czop B, Lis-Swiety A, Brzezińska-Wcisło L
BMC Neurol 2009 Jul 27;9:39. doi: 10.1186/1471-2377-9-39. PMID: 19635150Free PMC Article

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