MCID: GRN032
MIFTS: 28

Granulomatous Slack Skin Disease

Categories: Rare diseases, Skin diseases, Blood diseases

Aliases & Classifications for Granulomatous Slack Skin Disease

MalaCards integrated aliases for Granulomatous Slack Skin Disease:

Name: Granulomatous Slack Skin Disease 49 69
Granulomatous Slack Skin 49 55 51 69

Characteristics:

Orphanet epidemiological data:

55
granulomatous slack skin
Inheritance: Not applicable; Prevalence: <1/1000000 (Worldwide); Age of onset: Adult; Age of death: normal life expectancy;

Classifications:



Summaries for Granulomatous Slack Skin Disease

NIH Rare Diseases : 49 Granulomatous slack skin disease (GSS) is a very rare cutaneous disorder and less than 50 cases have been reported so far. The cause of this condition is unknown but it is often associated with mycosis fungiodes and other lymphoproliferative disorders such as Hodgkin and non-Hodgkin lymphoma. Signs and symptoms include loose hanging skin and fine wrinkling of the skin or papules. These symptoms are most often located in the axilla, groin and stomach. There is no specific treatment regimen for GSS. Treatment options are based on the recommendations for cutaneous T-cell lymphoma and include topical steroids, surgical remvoval of skin folds, radiation therapy and chemotherapy. Last updated: 1/24/2013

MalaCards based summary : Granulomatous Slack Skin Disease, also known as granulomatous slack skin, is related to skin disease and mycosis fungoides, and has symptoms including redundant skin, erythema and hodgkin lymphoma. An important gene associated with Granulomatous Slack Skin Disease is TRB (T Cell Receptor Beta Locus). The drugs Mechlorethamine and Triamcinolone have been mentioned in the context of this disorder. Affiliated tissues include skin, t cells and kidney.

Wikipedia : 72 Granulomatous slack skin (GSS) is a rare cutaneous condition, a variant of lymphoma that typically... more...

Related Diseases for Granulomatous Slack Skin Disease

Diseases related to Granulomatous Slack Skin Disease via text searches within MalaCards or GeneCards Suite gene sharing:

# Related Disease Score Top Affiliating Genes
1 skin disease 10.3
2 mycosis fungoides 10.0
3 autoimmune lymphoproliferative syndrome, type v 10.0

Symptoms & Phenotypes for Granulomatous Slack Skin Disease

Human phenotypes related to Granulomatous Slack Skin Disease:

55 31 (show all 10)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 redundant skin 55 31 frequent (33%) Frequent (79-30%) HP:0001582
2 erythema 55 31 frequent (33%) Frequent (79-30%) HP:0010783
3 hodgkin lymphoma 55 31 frequent (33%) Frequent (79-30%) HP:0012189
4 stiff skin 55 31 frequent (33%) Frequent (79-30%) HP:0030053
5 nephrocalcinosis 55 31 occasional (7.5%) Occasional (29-5%) HP:0000121
6 acute kidney injury 55 31 occasional (7.5%) Occasional (29-5%) HP:0001919
7 abnormality of the lymph nodes 55 31 occasional (7.5%) Occasional (29-5%) HP:0002733
8 hypercalcemia 55 31 occasional (7.5%) Occasional (29-5%) HP:0003072
9 cutis laxa 55 Frequent (79-30%)
10 lymphoma 55 Frequent (79-30%)

Drugs & Therapeutics for Granulomatous Slack Skin Disease

Drugs for Granulomatous Slack Skin Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 13)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Mechlorethamine Approved, Investigational Phase 2 51-75-2 4033
2
Triamcinolone Approved, Vet_approved Phase 2 124-94-7 31307
3 triamcinolone acetonide Phase 2
4 Alkylating Agents Phase 2
5 glucocorticoids Phase 2
6 Triamcinolone diacetate Phase 2
7 Triamcinolone hexacetonide Phase 2
8 Nitrogen Mustard Compounds Phase 2
9 Hormone Antagonists Phase 2
10 Hormones Phase 2
11 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2
12 Immunosuppressive Agents Phase 2
13 Anti-Inflammatory Agents Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Mechlorethamine Induced Contact Dermatitis Avoidance Study Recruiting NCT03380026 Phase 2 Triamcinolone;Valchlor 0.016 % Topical Gel

Search NIH Clinical Center for Granulomatous Slack Skin Disease

Genetic Tests for Granulomatous Slack Skin Disease

Anatomical Context for Granulomatous Slack Skin Disease

MalaCards organs/tissues related to Granulomatous Slack Skin Disease:

38
Skin, T Cells, Kidney, Lymph Node

Publications for Granulomatous Slack Skin Disease

Articles related to Granulomatous Slack Skin Disease:

# Title Authors Year
1
Granulomatous slack skin disease: a review, in comparison with mycosis fungoides. ( 22435618 )
2012
2
An unusual case of granulomatous slack skin disease with necrobiosis. ( 18806490 )
2008
3
Granulomatous slack skin disease in a child: the outcome. ( 18035987 )
2007
4
Granulomatous slack skin disease--disease features and response to pentostatin. ( 14531912 )
2003
5
A case of primary cutaneous CD30+ T-cell lymphoproliferative disorder with features of granulomatous slack skin disease. ( 12410714 )
2002

Variations for Granulomatous Slack Skin Disease

Expression for Granulomatous Slack Skin Disease

Search GEO for disease gene expression data for Granulomatous Slack Skin Disease.

Pathways for Granulomatous Slack Skin Disease

GO Terms for Granulomatous Slack Skin Disease

Sources for Granulomatous Slack Skin Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
Content
Loading form....