MCID: SCL002
MIFTS: 44

Scleredema Adultorum

Categories: Rare diseases, Skin diseases
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Aliases & Classifications for Scleredema Adultorum

MalaCards integrated aliases for Scleredema Adultorum:

Name: Scleredema Adultorum 11 19 43 14 16 71
Scleredema 19 58 75
Buschke's Scleredema 11 19
Scleredema Diabeticorum of Buschke 19
Scleredema Adultorum of Buschke 19
Scleredema Diabeticorum 19
Buschke Scleredema 58

Characteristics:


Age Of Onset:

Scleredema: Adolescent,Adult,Childhood,Infancy 58

Classifications:

Orphanet: 58  
Rare skin diseases


External Ids:

Disease Ontology 11 DOID:3140
MeSH 43 D012592
NCIt 49 C85057
SNOMED-CT 68 72967001
ICD10 via Orphanet 32 M34.8
Orphanet 58 ORPHA352763
UMLS 71 C0036413

Summaries for Scleredema Adultorum

Orphanet: 58 A rare acquired skin disease characterized by excessive mucin deposition and thickened collagen bundles in the dermis, resulting in woody, non-pitting induration of the skin of the neck, spreading to the shoulders and upper trunk, but sparing hands and feet. According to the association with preceding or underlying conditions, three types can be distinguished: type 1 usually follows a febrile infection, type 2 is associated with paraproteinemia, and type 3 occurs in patients with diabetes mellitus. Especially in types 2 and 3, extracutaneous involvement may be present. Other potentially associated conditions include a variety of endocrinopathies, systemic diseases, and neoplasms.

MalaCards based summary: Scleredema Adultorum, also known as scleredema, is related to graves disease 1 and systemic scleroderma, and has symptoms including skin hardening, pruritus and exanthema. An important gene associated with Scleredema Adultorum is INS (Insulin), and among its related pathways/superpathways are Signal Transduction and Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs). The drugs Histamine and Tranilast have been mentioned in the context of this disorder. Affiliated tissues include skin, bone marrow and lung, and related phenotypes are cellular and immune system

GARD: 19 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. In some cases, Scleredema resolves spontaneously on its own, while in other affected people, the condition persists for long periods of time.

Disease Ontology: 11 A skin disease that is characterized by induration of the skin, especially of the neck and upper trunk, has symptom skin hardening or stiffening, and is associated with infections (especially streptococcal), hematologic malignancies, diabetes, and adverse drug reactions.

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

Related Diseases for Scleredema Adultorum

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

(show top 50) (show all 247)
# Related Disease Score Top Affiliating Genes
1 graves disease 1 30.1 PTH POMC INS
2 systemic scleroderma 30.1 FN1 FBN1 CD8A
3 myxedema 29.8 POMC INS
4 cellulitis 29.8 INS CD8A CAMP
5 connective tissue disease 29.4 PTH MMP1 INS FN1 FBN1 CD8A
6 localized scleroderma 29.1 MMP1 FN1 FBN1 DCN CD8A
7 peripheral nervous system disease 28.8 POMC NCAM1 INS FN1 CD8A
8 skin disease 28.6 POMC NCAM1 MMP1 INS FN1 CXCL9
9 adducted thumbs syndrome 11.0
10 myeloma, multiple 10.3
11 raynaud disease 10.3
12 scleroderma, familial progressive 10.2
13 eosinophilic fasciitis 10.2
14 primary hyperparathyroidism 10.2
15 hyperparathyroidism 10.2
16 mucinoses 10.2
17 liver cirrhosis 10.2
18 fasciitis 10.2
19 alopecia 10.2
20 scleromyxedema 10.2
21 kidney hypertrophy 10.2 INS FN1
22 peyronie's disease 10.2 FN1 DCN
23 cervical incompetence 10.2 FN1 DCN
24 diffuse idiopathic skeletal hyperostosis 10.2 PTH INS
25 calciphylaxis 10.2 PTH FN1
26 hypertryptophanemia 10.2 NCAM1 INS
27 decubitus ulcer 10.2 INS FN1
28 tendinopathy 10.2 MMP1 DCN
29 nerve compression syndrome 10.2 PTH INS
30 isthmus cancer 10.2 PTH NCAM1
31 acidophil adenoma 10.2 POMC INS
32 cavernous sinus thrombosis 10.2 POMC INS
33 factitious disorder 10.2 POMC INS
34 hirata disease 10.2 POMC INS
35 pituitary infarct 10.2 POMC INS
36 central pontine myelinolysis 10.1 POMC INS
37 hypoglycemic coma 10.1 POMC INS
38 sheehan syndrome 10.1 POMC INS
39 hypothalamic disease 10.1 POMC INS
40 progressive familial heart block, type ia 10.1
41 stiff skin syndrome 10.1
42 ataxia with vitamin e deficiency 10.1
43 progressive familial heart block 10.1
44 rheumatic fever 10.1
45 pericarditis 10.1
46 amyloidosis 10.1
47 dextrocardia 10.1
48 follicular mucinosis 10.1
49 erythrokeratoderma ''en cocardes'' 10.1
50 lactose intolerance 10.1 PTH INS

Graphical network of the top 20 diseases related to Scleredema Adultorum:



Diseases related to Scleredema Adultorum

Symptoms & Phenotypes for Scleredema Adultorum

Symptoms:

11
  • skin hardening

UMLS symptoms related to Scleredema Adultorum:


pruritus; exanthema

MGI Mouse Phenotypes related to Scleredema Adultorum:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.91 CAMP CD8A DCN FBN1 FN1 INS
2 immune system MP:0005387 9.85 CAMP CD8A CXCL9 DCN FBN1 FN1
3 hematopoietic system MP:0005397 9.61 CAMP CD8A CXCL9 DCN FBN1 FN1
4 mortality/aging MP:0010768 9.32 CAMP CD8A CXCL9 DCN FBN1 FN1

Drugs & Therapeutics for Scleredema Adultorum

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

(show all 17)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Histamine Approved, Investigational Early Phase 1 51-45-6 774
2
Tranilast Investigational Early Phase 1 53902-12-8 5282230
3 Analgesics Early Phase 1
4 Antirheumatic Agents Early Phase 1
5 Neurotransmitter Agents Early Phase 1
6 Hormones Early Phase 1
7 Anti-Allergic Agents Early Phase 1
8 Calcium, Dietary Early Phase 1
9 Platelet Aggregation Inhibitors Early Phase 1
10
Histamine phosphate Early Phase 1 51-74-1 134614
11 Anti-Inflammatory Agents, Non-Steroidal Early Phase 1
12 calcium channel blockers Early Phase 1
13 Histamine H1 Antagonists Early Phase 1
14 Histamine Antagonists Early Phase 1
15 Analgesics, Non-Narcotic Early Phase 1
16 Anti-Inflammatory Agents Early Phase 1
17
Calcium Nutraceutical Early Phase 1 7440-70-2 271

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Clinical Study of Tranilast in the Treatment of Scleredema Diabeticorum Unknown status NCT03512873 Early Phase 1 Tranilast
2 Screening Patients With Diabetes Mellitus for the Presence of Scleredema Adultorum of Buschke and Characterize Clinical-laboratory Findings of the Newly Identified Cases: a Single-center, Prospective, Obsevational Study Recruiting NCT04335396

Search NIH Clinical Center for Scleredema Adultorum

Cochrane evidence based reviews: scleredema adultorum

Genetic Tests for Scleredema Adultorum

Anatomical Context for Scleredema Adultorum

Organs/tissues related to Scleredema Adultorum:

MalaCards : Skin, Bone Marrow, Lung, Pituitary, Thyroid, Breast, Heart

Publications for Scleredema Adultorum

Articles related to Scleredema Adultorum:

(show top 50) (show all 425)
# Title Authors PMID Year
1
Recurrent Generalized Scleredema in an Adolescent Girl With Uncontrolled Type 1 Diabetes Mellitus. 62
36370020 2022
2
Indurated plaques on the back: distinguishing stiff skin syndrome from scleredema and morphea. 62
34411278 2022
3
New and emerging conditions of acquired cutaneous mucinoses in adults. 62
35124832 2022
4
Scleredema developing after SARS-CoV-2 infection: a possible novel skin manifestation of coronavirus disease. 62
35514098 2022
5
Usefulness of high-frequency Doppler ultrasound skin thickness measurement for disease staging and assessing treatment response in patients with scleredema: A case-control study. 62
33493571 2022
6
Monoclonal Gammopathy-Associated Scleredema Adultorum of Buschke in a Patient with Diabetes Mellitus Successfully Treated with Intravenous Immunoglobulin and Narrow-Band Ultraviolet B Phototherapy: A Case Report. 62
34858014 2021
7
[Scleredema: Beyond the hardening of the skin]. 62
34154911 2021
8
Scleredema with biopsy-confirmed cardiomyopathy: A case report. 62
35387215 2021
9
Scleredema adultorum of Buschke associated with hypothyroidism and liver cirrhosis. 62
34380804 2021
10
Efficacy of vitamin E combined with heparin sodium cream in treatment of scleredema neonatorum. 62
31355597 2021
11
Scleredema diabeticorum in a patient: un uncommon etiology of restrictive lung pattern Escleredema diabeticorum en un paciente: una etiología infrecuente de patrón restrictivo pulmonar. 62
33865279 2021
12
Scleredema diabeticorum - A case report. 62
34041118 2021
13
Cutaneous Manifestations of Nutritional Excess: Pathophysiologic Effects of Hyperglycemia and Hyperinsulinemia on the Skin. 62
33891835 2021
14
Early Physical Linear Growth of Small-for-Gestational-Age Infants Based on Computer Analysis Method. 62
34697568 2021
15
Unilateral Linear Induration of the Skin: A Case Report of an Unusual Presentation of Scleredema. 62
33488921 2020
16
Concurrent Scleredema and Pyoderma Gangrenosum: Case Report and Review of Comorbid Conditions. 62
33489598 2020
17
Diabetes-associated dermatological manifestations in primary care and their association with vascular complications. 62
33520817 2020
18
Scleredema and nodal inflammatory pseudotumor: IgG4-related disease? 62
32530044 2020
19
Hyaluronidase in Dermatology: Uses Beyond Hyaluronic Acid Fillers. 62
33026763 2020
20
A case of paraproteinemia-associated scleredema successfully treated with thalidomide. 62
32995440 2020
21
Scleredema of Buschke associated with lichen sclerosus: Three cases. 62
30289118 2020
22
Scleredema diabeticorum: Description of 11 cases. 62
30717999 2020
23
Scleredema adultorum of Buschke treated by extracorporeal shock wave therapy. 62
31733081 2020
24
Sclerodermalike syndromes: Great imitators. 62
32513403 2020
25
Scleredema in the setting of Rheumatoid Arthritis. 62
31502275 2020
26
A case of concurrent systemic sclerosis and scleredema. 62
31687459 2019
27
[Sclerosing skin diseases]. 62
31292667 2019
28
A comparative study of thoracoscopic and open surgery of congenital diaphragmatic hernia in neonates. 62
31242917 2019
29
New insights on scleromyxedema. 62
35382389 2019
30
Intravenous immunoglobulin treatment: Where do dermatologists stand? 62
30756448 2019
31
Scleredema associated with immunoglobulin A-κ smoldering myeloma: a case report and review of the literature. 62
31084620 2019
32
Reticular Erythematous Mucinosis: A Rare Cutaneous. 62
31032786 2019
33
Coexisting cutaneous macroglobulinosis and scleredema of Buschke in a patient with a Waldenström Macroglobulinemia. 62
30267590 2019
34
Youth-Onset Type 2 Diabetes Manifestations in other Specialties: Its Many Disguises. 62
31096219 2019
35
An Unusual Presentation of a Rare Scleroderma Mimic: What is Behind the Scenes? 62
30088450 2019
36
Implementation and evaluation of a rehabilitation concept in a patient suffering from Scleredema Adultorum Buschke: a case report. 62
28738698 2018
37
[Efficacy and safety of selective brain hypothermia therapy on neonatal hypoxic-ischemic encephalopathy]. 62
30541643 2018
38
[Scleredema adultorum : Clinical presentation, diagnostic workup, differential diagnosis, treatment option]. 62
30128796 2018
39
Paclitaxel-related scleredema-like skin changes in a patient with breast cancer. 62
28944958 2018
40
Serositis in a child with scleredema of Buschke: an unusual association and improvement following glucocorticoids. 62
28895446 2018
41
Ultrasonographic Findings of Scleredema Adultorum of Buschke Involving the Posterior Neck. 62
29713220 2018
42
[Buschke's scleredema successfully treated with extracorporeal photopheresis]. 62
29544653 2018
43
Successful Treatment of Scleredema Diabeticorum With Tranilast: Three Case Reports. 62
29374068 2018
44
Scleroderma-like Disorders. 62
28606036 2018
45
Scleroderma with an update about clinico-pathological correlation. 62
29368844 2018
46
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'. 62
29283443 2018
47
Scleredema Diabeticorum with Superimposed Cellulitis and Abscess Formation. 62
29850291 2018
48
Anti-carbamylated protein antibodies and skin involvement in patients with systemic sclerosis: An intriguing association. 62
30596753 2018
49
Miscellaneous skin disease and the metabolic syndrome. 62
29241760 2018
50
Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review. 62
28712039 2017

Variations for Scleredema Adultorum

Expression for Scleredema Adultorum

Search GEO for disease gene expression data for Scleredema Adultorum.

Pathways for Scleredema Adultorum

GO Terms for Scleredema Adultorum

Cellular components related to Scleredema Adultorum according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.86 PTH POMC INS FN1 FBN1 DCN
2 extracellular region GO:0005576 9.66 PTH POMC NCAM1 MMP1 INS FN1

Biological processes related to Scleredema Adultorum according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of glycogen biosynthetic process GO:0045725 9.46 PTH INS
2 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.43 INS FN1 DCN
3 cell-cell signaling GO:0007267 9.23 PTH POMC INS CXCL9

Molecular functions related to Scleredema Adultorum according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 hormone activity GO:0005179 9.23 PTH POMC INS FBN1

Sources for Scleredema Adultorum

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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