MCID: SCL022
MIFTS: 31

Scleredema

Categories: Rare diseases, Skin diseases

Aliases & Classifications for Scleredema

MalaCards integrated aliases for Scleredema:

Name: Scleredema 53 59
Scleredema Adultorum 53 72
Scleredema Diabeticorum of Buschke 53
Scleredema Adultorum of Buschke 53
Scleredema Diabeticorum 53
Buschke's Scleredema 53
Buschke Scleredema 59

Characteristics:

Orphanet epidemiological data:

59
scleredema
Inheritance: Not applicable; Age of onset: Adolescent,Adult,Childhood,Infancy;

Classifications:

Orphanet: 59  
Rare skin diseases


External Ids:

ICD10 via Orphanet 34 M34.8
Orphanet 59 ORPHA352763
UMLS 72 C0036413

Summaries for Scleredema

NIH Rare Diseases : 53 Scleredema is a form of cutaneous mucinosis, a diverse group of rare skin conditions that are characterized by an accumulation of mucin (a jelly-like complex carbohydrate substance) in the skin. Signs and symptoms of this condition include hardening and thickening of the skin which may restrict movement. Skin in affected areas may be red or brown and often has an 'orange-skin' appearance. There are three forms of the condition which vary by disease course and long term outlook (prognosis). Although the underlying cause is currently unknown, each form is associated with a different condition: infection (type 1), blood abnormalities (type 2), and diabetes (type 3). In some cases, scleredema resolves spontaneously on its own, while in other affected people, the condition persists for long periods of time. Due to the rarity of the condition, there is no standard treatment.

MalaCards based summary : Scleredema, also known as scleredema adultorum, is related to scleredema adultorum and systemic scleroderma, and has symptoms including pruritus and exanthema. The drugs Histamine and Calcium have been mentioned in the context of this disorder. Affiliated tissues include skin, testes and brain.

Wikipedia : 75 Scleredema, is a rare, self-limiting skin condition defined by progressive thickening and hardening of... more...

Related Diseases for Scleredema

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

(show top 50) (show all 115)
# Related Disease Score Top Affiliating Genes
1 scleredema adultorum 12.6
2 systemic scleroderma 10.5
3 myeloma, multiple 10.5
4 diabetes mellitus 10.4
5 skin disease 10.3
6 scleromyxedema 10.3
7 scleroderma, familial progressive 10.3
8 eosinophilic fasciitis 10.3
9 hydrops, lactic acidosis, and sideroblastic anemia 10.3
10 fasciitis 10.3
11 amyloidosis 10.2
12 mucinoses 10.1
13 alopecia 10.1
14 raynaud phenomenon 10.1
15 acanthosis nigricans 10.0
16 atrial standstill 1 10.0
17 hypercholesterolemia, familial, 1 10.0
18 dowling-degos disease 1 10.0
19 rheumatoid arthritis 10.0
20 stiff skin syndrome 10.0
21 kearns-sayre syndrome 10.0
22 primary hyperparathyroidism 10.0
23 hyperparathyroidism 10.0
24 familial hypercholesterolemia 10.0
25 polyneuropathy 10.0
26 necrobiosis lipoidica 10.0
27 cellulitis 10.0
28 connective tissue disease 10.0
29 localized scleroderma 10.0
30 arthritis 10.0
31 neuropathy 10.0
32 smoldering myeloma 10.0
33 nephrogenic systemic fibrosis 10.0
34 spondyloarthropathy 1 9.9
35 apnea, obstructive sleep 9.9
36 autoimmune disease 9.9
37 gastroesophageal reflux 9.9
38 progressive familial heart block, type ia 9.9
39 diabetes mellitus, noninsulin-dependent 9.9
40 lichen sclerosus et atrophicus 9.9
41 myositis 9.9
42 pernicious anemia 9.9
43 hutchinson-gilford progeria syndrome 9.9
44 vitiligo-associated multiple autoimmune disease susceptibility 6 9.9
45 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 9.9
46 myelofibrosis 9.9
47 myxedema 9.9
48 ocular motor apraxia 9.9
49 rheumatic fever-related antigen 9.9
50 body mass index quantitative trait locus 11 9.9

Graphical network of the top 20 diseases related to Scleredema:



Diseases related to Scleredema

Symptoms & Phenotypes for Scleredema

UMLS symptoms related to Scleredema:


pruritus, exanthema

Drugs & Therapeutics for Scleredema

Drugs for Scleredema (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 18)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Histamine Approved, Investigational Early Phase 1 51-45-6 774
2
Calcium Approved, Nutraceutical Early Phase 1 7440-70-2 271
3
Tranilast Investigational Early Phase 1 53902-12-8 93543
4 Neurotransmitter Agents Early Phase 1
5 Hormones Early Phase 1
6 Anti-Allergic Agents Early Phase 1
7 Analgesics, Non-Narcotic Early Phase 1
8 Peripheral Nervous System Agents Early Phase 1
9 Analgesics Early Phase 1
10 Anti-Inflammatory Agents, Non-Steroidal Early Phase 1
11 Histamine Antagonists Early Phase 1
12 Histamine H1 Antagonists Early Phase 1
13 Platelet Aggregation Inhibitors Early Phase 1
14
Histamine Phosphate Early Phase 1 51-74-1 65513
15 Anti-Inflammatory Agents Early Phase 1
16 Calcium, Dietary Early Phase 1
17 calcium channel blockers Early Phase 1
18 Antirheumatic Agents Early Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Clinical Study of Tranilast in the Treatment of Scleredema Diabeticorum Not yet recruiting NCT03512873 Early Phase 1 Tranilast

Search NIH Clinical Center for Scleredema

Genetic Tests for Scleredema

Anatomical Context for Scleredema

MalaCards organs/tissues related to Scleredema:

41
Skin, Testes, Brain, Heart, Lung, Bone, Bone Marrow

Publications for Scleredema

Articles related to Scleredema:

(show top 50) (show all 400)
# Title Authors PMID Year
1
[Sclerosing skin diseases]. 38
31292667 2019
2
Efficacy of vitamin E combined with heparin sodium cream in treatment of scleredema neonatorum. 38
31355597 2019
3
A comparative study of thoracoscopic and open surgery of congenital diaphragmatic hernia in neonates. 38
31242917 2019
4
Scleredema associated with immunoglobulin A-κ smoldering myeloma: a case report and review of the literature. 38
31084620 2019
5
Intravenous immunoglobulin treatment: Where do dermatologists stand? 38
30756448 2019
6
Reticular Erythematous Mucinosis: A Rare Cutaneous. 38
31032786 2019
7
Coexisting cutaneous macroglobulinosis and scleredema of Buschke in a patient with a Waldenström Macroglobulinemia. 38
30267590 2019
8
Scleredema diabeticorum: Description of 11 cases. 38
30717999 2019
9
An Unusual Presentation of a Rare Scleroderma Mimic: What is Behind the Scenes? 38
30088450 2019
10
Youth-Onset Type 2 Diabetes Manifestations in other Specialties: Its Many Disguises. 38
31096219 2019
11
[Scleredema adultorum : Clinical presentation, diagnostic workup, differential diagnosis, treatment option]. 38
30128796 2018
12
Implementation and evaluation of a rehabilitation concept in a patient suffering from Scleredema Adultorum Buschke: a case report. 38
28738698 2018
13
[Efficacy and safety of selective brain hypothermia therapy on neonatal hypoxic-ischemic encephalopathy]. 38
30541643 2018
14
Scleredema of Buschke associated with lichen sclerosus: Three cases. 38
30289118 2018
15
Paclitaxel-related scleredema-like skin changes in a patient with breast cancer. 38
28944958 2018
16
Serositis in a child with scleredema of Buschke: an unusual association and improvement following glucocorticoids. 38
28895446 2018
17
[Buschke's scleredema successfully treated with extracorporeal photopheresis]. 38
29544653 2018
18
Ultrasonographic Findings of Scleredema Adultorum of Buschke Involving the Posterior Neck. 38
29713220 2018
19
Scleroderma with an update about clinico-pathological correlation. 38
29368844 2018
20
Scleroderma-like Disorders. 38
28606036 2018
21
Successful Treatment of Scleredema Diabeticorum With Tranilast: Three Case Reports. 38
29374068 2018
22
Letter on 'European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis'. 38
29283443 2018
23
Miscellaneous skin disease and the metabolic syndrome. 38
29241760 2018
24
Anti-carbamylated protein antibodies and skin involvement in patients with systemic sclerosis: An intriguing association. 38
30596753 2018
25
Scleredema Diabeticorum with Superimposed Cellulitis and Abscess Formation. 38
29850291 2018
26
Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review. 38
28712039 2017
27
[SKIN PATHOLOGY IN DIABETES MELLITUS: CLINICAL AND PATHOPHYSIOLOGICAL CORRELATIONS (REVIEW)]. 38
29328028 2017
28
European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis. 38
28786499 2017
29
Scleredema of Buschke with prominent periorbital edema. 38
28464273 2017
30
Cutaneous Manifestations of Diabetes Mellitus: A Review. 38
28374407 2017
31
Successful treatment with IVIg therapy of diabetes-associated scleredema severe progressive case and review of the literature. 38
28573670 2017
32
Pathophysiological Mechanisms in Sclerosing Skin Diseases. 38
28868289 2017
33
Idiopathic scleredema. 38
28329532 2016
34
Scleredema of Buschke. 38
28149667 2016
35
Atypical Scleredema Involving the Hands in an Adolescent. 38
27573162 2016
36
Laboratory Testing in the Setting of Diabetic Scleredema. 38
27483073 2016
37
Laboratory Testing in the Setting of Diabetic Scleredema--Reply. 38
27483076 2016
38
[Risk factors for concurrent sepsis in neonates with necrotizing enterocolitis]. 38
27530780 2016
39
Scleredema of Buschke associated with difficult-to-control type 2 diabetes mellitus. 38
27310540 2016
40
Scleredema adultorum of Buschke with prominent periorbital edema in a Tunisian patient with diabetes mellitus: a case report. 38
26536602 2016
41
A case of scleredema adultorum successfully treated with narrow-band ultraviolet B phototherapy. 38
24499427 2016
42
Long-term benefit of electron beam radiation therapy in the treatment of scleredema of Buschke. 38
28799569 2016
43
Therapy resistant idiopathic scleredema: an underlying pathology not always present. 38
27708956 2015
44
Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients. 38
26304054 2015
45
[Monoclonal immunoglobulin (M-Ig) and skin diseases from the group of mucinoses--scleredema adultorum Buschke and scleromyxedema. Description of four cases and an overview of therapies]. 38
26806503 2015
46
Prevalence of Skin Changes in Diabetes Mellitus and its Correlation with Internal Diseases: A Single Center Observational Study. 38
26538693 2015
47
Scleredema of Buschke Following Streptococcal Infection. 38
26273177 2015
48
Scleredema Diabeticorum - A Case Report. 38
26329963 2015
49
Clinical correlates of human leucocyte antigen (HLA)-G in systemic sclerosis. 38
25847615 2015
50
Scleredema associated with Sjögren's syndrome. 38
26312681 2015

Variations for Scleredema

Expression for Scleredema

Search GEO for disease gene expression data for Scleredema.

Pathways for Scleredema

GO Terms for Scleredema

Sources for Scleredema

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
Content
Loading form....