MCID: LNR005
MIFTS: 22

Linear Scleroderma

Categories: Rare diseases, Skin diseases

Aliases & Classifications for Linear Scleroderma

MalaCards integrated aliases for Linear Scleroderma:

Name: Linear Scleroderma 52 71 32
Coup De Sabre Scleroderma 71
Scleroderma, Linear 52
En Coup De Sabre 52

Classifications:



External Ids:

ICD10 32 L94.1
UMLS 71 C0263409 C0392441

Summaries for Linear Scleroderma

NIH Rare Diseases : 52 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 cerebellum.

Wikipedia : 74 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 138)
# 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 lichen sclerosus et atrophicus 10.4
7 discoid lupus erythematosus 10.4
8 melorheostosis, isolated 10.3
9 scleroderma, familial progressive 10.2
10 adie pupil 10.2
11 coloboma of macula 10.2
12 hair whorl 10.2
13 nevus, epidermal 10.2
14 rheumatoid arthritis 10.2
15 sturge-weber syndrome 10.2
16 torticollis 10.2
17 trigeminal neuralgia 10.2
18 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.2
19 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 10.2
20 pars planitis 10.2
21 intraocular pressure quantitative trait locus 10.2
22 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 10.2
23 cutaneous lupus erythematosus 10.2
24 bone resorption disease 10.2
25 migraine with aura 10.2
26 gingival recession 10.2
27 lyme disease 10.2
28 primary hypertrophic osteoarthropathy 10.2
29 atrophic glossitis 10.2
30 status epilepticus 10.2
31 focal epilepsy 10.2
32 movement disease 10.2
33 aortic valve insufficiency 10.2
34 ocular hypotension 10.2
35 lung disease 10.2
36 herpes zoster 10.2
37 cleft lip 10.2
38 cutaneous sclerosis 10.2
39 diffuse cutaneous systemic sclerosis 10.2
40 herpes zoster ophthalmicus 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 ptosis 10.2
49 eosinophilic fasciitis 10.2
50 connective tissue disease 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:

40
Skin, Brain, Cerebellum, Heart, Thyroid

Publications for Linear Scleroderma

Articles related to Linear Scleroderma:

(show top 50) (show all 366)
# Title Authors PMID Year
1
[Linear scleroderma "en coup de sabre" causing severe neurological and ocular symptoms]. 61
32533203 2020
2
Ultraviolet A1 phototherapy for the treatment of localized scleroderma. 61
32383187 2020
3
Methotrexate in Linear Scleroderma: long-term efficacy in 50 children from a single Pediatric Rheumatology Centre. 61
32433808 2020
4
Treatment of linear scleroderma "En coup de Sabre" with single-stage autologous fat grafting: A case report and review of the literature. 61
32426912 2020
5
Overview of Juvenile localized scleroderma and its management. 61
31786801 2020
6
A case of overlapping adult-onset linear scleroderma and Parry-Romberg syndrome presenting with widespread ipsilateral neurogenic involvement. 61
31775183 2020
7
Extensive linear scleroderma en coup de sabre with exertion-induced hemiplegic migraine. 61
31670009 2020
8
A case of multiple cerebral cavernomas associated with en coup de sabre scleroderma. 61
31755138 2020
9
A case of linear scleroderma "en coup de sabre" with strabismus fixus in a child. 61
31544571 2019
10
Characteristics of coexisting localized scleroderma and inflammatory arthritis. 61
31804172 2019
11
Linear Patch of Alopecia in a Child: Trichoscopy Reveals the Actual Diagnosis. 61
31799275 2019
12
Acquired Strabismus in Linear Scleroderma of the Face. 61
31197382 2019
13
Association between frontal fibrosing alopecia and linear scleroderma "coup de sabre". 61
30613936 2019
14
Diagnosis and management of linear scleroderma in children. 61
31157654 2019
15
[Orthopedic management of localized linear scleroderma in children: a case report]. 61
32246599 2019
16
Interventions for morphea. 61
31309547 2019
17
Linear scleroderma "en coup de sabre" with extensive brain involvement-Clinicopathologic correlations and response to anti-Interleukin-6 therapy. 61
31096996 2019
18
Parry-Romberg syndrome associated with en coup de sabre in a patient from South Sudan - a rare entity from East Africa: a case report. 61
31046814 2019
19
Retinal vasculitis the first clue in the diagnosis of progressive hemifacial atrophy. 61
31329538 2019
20
Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry. 61
31777788 2019
21
A surgical approach to linear scleroderma using Medpor and dermal fat graft. 61
30332890 2019
22
Linear lipoatrophy following intra-articular triamcinolone acetonide injection mimicking linear scleroderma. 61
30484902 2019
23
A queer case of Parry-Romberg syndrome with coexisting psychiatric ailment. 61
30745662 2019
24
New Features for Measuring Disease Activity in Pediatric Localized Scleroderma. 61
30219769 2018
25
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. 61
30168188 2018
26
Ophthalmic findings in linear scleroderma manifesting as facial en coup de sabre. 61
29973692 2018
27
A 26-Year Experience with Microsurgical Reconstruction of Hemifacial Atrophy and Linear Scleroderma. 61
30511981 2018
28
Treatments and outcomes in juvenile linear scleroderma: a narrative systematic review. 61
30442631 2018
29
Disease course and long-term outcome of juvenile localized scleroderma: Experience from a single pediatric rheumatology Centre and literature review. 61
29729451 2018
30
Focal Epilepsy in a Teenager With Facial Atrophy and Hair Loss. 61
29961525 2018
31
Histopathologic Findings of Linear Scleroderma Displaying Focal Trichiasis Secondary to Tarsal Thinning. 61
29851754 2018
32
Neuroimaging and clinical findings in a case of linear scleroderma en coup de sabre. 61
29849855 2018
33
Trichoscopic Features of Linear Morphea on the Scalp. 61
29457011 2018
34
Characterization and Treatment of Unilateral Facial Muscle Spasm in Linear Scleroderma: A Case Report. 61
29416936 2018
35
Cone beam computed tomography for the assessment of linear scleroderma of the face. 61
29298697 2018
36
Lacrimal Drainage System Involvement in Linear Scleroderma. 61
27930422 2017
37
Craniofacial linear scleroderma associated with retinal telangiectasia and exudative retinal detachment. 61
28528991 2017
38
A case of systemic sclerosis with linear scleroderma developing after fracture surgery. 61
28251893 2017
39
Parry Romberg Syndrome and linear facial scleroderma: management in pediatric population. 61
28691464 2017
40
Morphea "en coup de sabre": An unusual oral presentation. 61
28210455 2017
41
Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease? 61
29445726 2017
42
[Facial linear scleroderma associated with neurological abnormalities relating to microangiopathy]. 61
27663385 2016
43
Linear scleroderma en coup de sabre presenting with positional diplopia and enophthalmos. 61
27754909 2016
44
Nummular Headache Associated With Linear Scleroderma. 61
27477768 2016
45
Linear scleroderma with prominent multiple lymphadenopathy followed by the development of polymyositis: A case report and review of published work. 61
27129435 2016
46
Treatment of Linear Scleroderma (en Coup de Sabre) With Dermal Fat Grafting. 61
26700540 2016
47
Imaging and clinical findings in a case of linear scleroderma en coup de sabre. 61
30460009 2016
48
Facial asymmetry: a case report of localized linear scleroderma patient with muscular strain and spasm. 61
26413496 2015
49
A rare case of juvenile localised scleroderma with intra-oral and dental involvement. 61
26668618 2015
50
Linear atrophoderma of Moulin: an underrecognized entity. 61
26438123 2015

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 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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