MCID: LNR005
MIFTS: 23

Linear Scleroderma

Categories: Rare diseases, Skin diseases

Aliases & Classifications for Linear Scleroderma

MalaCards integrated aliases for Linear Scleroderma:

Name: Linear Scleroderma 53 72 33
Coup De Sabre Scleroderma 72
Scleroderma, Linear 53
En Coup De Sabre 53

Classifications:



External Ids:

ICD10 33 L94.1
UMLS 72 C0263409 C0392441

Summaries for Linear Scleroderma

NIH Rare Diseases : 53 Linear scleroderma is one sub-type of localized scleroderma, most commonly occurring in childhood. It is characterized by abnormalities of the skin and subcutaneous tissues that often follow a dermatomal distribution and that are found on one side of the body. Besides the lesion in the face or scalp there are also abnormalities of the muscles, fat tissue and skull. When the face is affected, some strips located on the forehead may be hollow and lead to an appearance termed "en coup de sabre". In most cases, Raynaud's phenomenon is absent. The exact cause is still unknown but may be related to an autoimmune reaction resulting in too much collagen. Management is symptomatic and includes immunosupressant medication. Physical therapy is helpful for the muscle retraction problems.

MalaCards based summary : Linear Scleroderma, also known as coup de sabre scleroderma, is related to hemifacial atrophy, progressive and systemic scleroderma. Affiliated tissues include skin, brain and heart.

Wikipedia : 75 Morphea, is a form of scleroderma that involves isolated patches of hardened skin on the face, hands,... more...

Related Diseases for Linear Scleroderma

Diseases related to Linear Scleroderma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 140)
# Related Disease Score Top Affiliating Genes
1 hemifacial atrophy, progressive 11.9
2 systemic scleroderma 11.8
3 localized scleroderma 11.7
4 melorheostosis 11.7
5 facial hemiatrophy 10.5
6 capillary malformations, congenital 10.4
7 melorheostosis, isolated 10.3
8 adie pupil 10.2
9 coloboma of macula 10.2
10 hair whorl 10.2
11 nevus, epidermal 10.2
12 rheumatoid arthritis 10.2
13 sturge-weber syndrome 10.2
14 torticollis 10.2
15 trigeminal neuralgia 10.2
16 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.2
17 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 10.2
18 pars planitis 10.2
19 intraocular pressure quantitative trait locus 10.2
20 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 10.2
21 bone resorption disease 10.2
22 migraine with aura 10.2
23 gingival recession 10.2
24 lyme disease 10.2
25 primary hypertrophic osteoarthropathy 10.2
26 atrophic glossitis 10.2
27 status epilepticus 10.2
28 epilepsy 10.2
29 focal epilepsy 10.2
30 movement disease 10.2
31 aortic valve insufficiency 10.2
32 ocular hypotension 10.2
33 lung disease 10.2
34 herpes zoster 10.2
35 cleft lip 10.2
36 cutaneous sclerosis 10.2
37 diffuse cutaneous systemic sclerosis 10.2
38 herpes zoster ophthalmicus 10.2
39 lichen planopilaris 10.2
40 lichen sclerosus 10.2
41 pediatric multiple sclerosis 10.2
42 rhabdomyomatous mesenchymal hamartoma 10.2
43 weber syndrome 10.2
44 encephalopathy 10.2
45 hypertonia 10.2
46 hypotonia 10.2
47 systemic lupus erythematosus 10.2
48 scleroderma, familial progressive 10.2
49 eosinophilic fasciitis 10.2
50 ptosis 10.2

Graphical network of the top 20 diseases related to Linear Scleroderma:



Diseases related to Linear Scleroderma

Symptoms & Phenotypes for Linear Scleroderma

Drugs & Therapeutics for Linear Scleroderma

Search Clinical Trials , NIH Clinical Center for Linear Scleroderma

Genetic Tests for Linear Scleroderma

Anatomical Context for Linear Scleroderma

MalaCards organs/tissues related to Linear Scleroderma:

41
Skin, Brain, Heart, Thyroid, Cerebellum

Publications for Linear Scleroderma

Articles related to Linear Scleroderma:

(show top 50) (show all 353)
# Title Authors PMID Year
1
Diagnosis and management of linear scleroderma in children. 38
31157654 2019
2
Association between frontal fibrosing alopecia and linear scleroderma "coup de sabre". 38
30613936 2019
3
Interventions for morphea. 38
31309547 2019
4
Acquired Strabismus in Linear Scleroderma of the Face. 38
31197382 2019
5
Parry-Romberg syndrome associated with en coup de sabre in a patient from South Sudan - a rare entity from East Africa: a case report. 38
31046814 2019
6
Retinal vasculitis the first clue in the diagnosis of progressive hemifacial atrophy. 38
31329538 2019
7
Linear scleroderma "en coup de sabre" with extensive brain involvement-Clinicopathologic correlations and response to anti-Interleukin-6 therapy. 38
31096996 2019
8
A surgical approach to linear scleroderma using Medpor and dermal fat graft. 38
30332890 2019
9
A queer case of Parry-Romberg syndrome with coexisting psychiatric ailment. 38
30745662 2019
10
Linear lipoatrophy following intra-articular triamcinolone acetonide injection mimicking linear scleroderma. 38
30484902 2019
11
New Features for Measuring Disease Activity in Pediatric Localized Scleroderma. 38
30219769 2018
12
A 26-Year Experience with Microsurgical Reconstruction of Hemifacial Atrophy and Linear Scleroderma. 38
30511981 2018
13
Remission of seizures with immunosuppressive therapy in Parry-Romberg syndrome and en coup de sabre linear scleroderma: Case report and brief review of the literature. 38
30168188 2018
14
Ophthalmic findings in linear scleroderma manifesting as facial en coup de sabre. 38
29973692 2018
15
Treatments and outcomes in juvenile linear scleroderma: a narrative systematic review. 38
30442631 2018
16
Disease course and long-term outcome of juvenile localized scleroderma: Experience from a single pediatric rheumatology Centre and literature review. 38
29729451 2018
17
Focal Epilepsy in a Teenager With Facial Atrophy and Hair Loss. 38
29961525 2018
18
Histopathologic Findings of Linear Scleroderma Displaying Focal Trichiasis Secondary to Tarsal Thinning. 38
29851754 2018
19
Neuroimaging and clinical findings in a case of linear scleroderma en coup de sabre. 38
29849855 2018
20
Trichoscopic Features of Linear Morphea on the Scalp. 38
29457011 2018
21
Cone beam computed tomography for the assessment of linear scleroderma of the face. 38
29298697 2018
22
Characterization and Treatment of Unilateral Facial Muscle Spasm in Linear Scleroderma: A Case Report. 38
29416936 2018
23
Lacrimal Drainage System Involvement in Linear Scleroderma. 38
27930422 2017
24
Craniofacial linear scleroderma associated with retinal telangiectasia and exudative retinal detachment. 38
28528991 2017
25
A case of systemic sclerosis with linear scleroderma developing after fracture surgery. 38
28251893 2017
26
Parry Romberg Syndrome and linear facial scleroderma: management in pediatric population. 38
28691464 2017
27
Morphea "en coup de sabre": An unusual oral presentation. 38
28210455 2017
28
Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease? 38
29445726 2017
29
[Facial linear scleroderma associated with neurological abnormalities relating to microangiopathy]. 38
27663385 2016
30
Linear scleroderma with prominent multiple lymphadenopathy followed by the development of polymyositis: A case report and review of published work. 38
27129435 2016
31
Linear scleroderma en coup de sabre presenting with positional diplopia and enophthalmos. 38
27754909 2016
32
Nummular Headache Associated With Linear Scleroderma. 38
27477768 2016
33
Treatment of Linear Scleroderma (en Coup de Sabre) With Dermal Fat Grafting. 38
26700540 2016
34
Imaging and clinical findings in a case of linear scleroderma en coup de sabre. 38
30460009 2016
35
Facial asymmetry: a case report of localized linear scleroderma patient with muscular strain and spasm. 38
26413496 2015
36
A rare case of juvenile localised scleroderma with intra-oral and dental involvement. 38
26668618 2015
37
Linear atrophoderma of Moulin: an underrecognized entity. 38
26438123 2015
38
Overlap between linear scleroderma, progressive facial hemiatrophy and immune-inflammatory encephalitis in a paediatric cohort. 38
25851111 2015
39
Linear scleroderma associated with neurofibromatosis type I. 38
26367758 2015
40
[Localized Scleroderma of Lower Extremities:Clinical and Magnetic Resonance Imaging Features]. 38
26564453 2015
41
Pediatric linear scleroderma initially developed with angioma serpiginosum-like appearances. 38
25959512 2015
42
Linear scleroderma en coup de sabre including abnormal dental development. 38
25355303 2015
43
Progressive hemifacial atrophy: a review. 38
25881068 2015
44
Delay in Diagnosis of Congenital Linear Scleroderma until Adulthood. 38
25775624 2015
45
Atypical Neuroimaging Manifestations of Linear Scleroderma "en coup de sabre". 38
26401155 2015
46
A Case Report of Successful Treatment of Recalcitrant Childhood Localized Scleroderma with Infliximab and Leflunomide. 38
26161155 2015
47
Autologous fat transplantation for the treatment of linear scleroderma en coup de sabre. 38
25842477 2015
48
Use of a blunt-tipped microcannula for soft tissue filler injection in the treatment of linear scleroderma (en coup de sabre). 38
25361200 2014
49
Delay in Diagnosis of Congenital Linear Scleroderma until Adulthood. 38
25278149 2014
50
Hair disorders associated with autoimmune connective tissue diseases. 38
24975949 2014

Variations for Linear Scleroderma

Expression for Linear Scleroderma

Search GEO for disease gene expression data for Linear Scleroderma.

Pathways for Linear Scleroderma

GO Terms for Linear Scleroderma

Sources for Linear Scleroderma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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