MCID: GRN032
MIFTS: 29

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 53 73
Granulomatous Slack Skin 53 59 55 73

Characteristics:

Orphanet epidemiological data:

59
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 : 53 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.

MalaCards based summary : Granulomatous Slack Skin Disease, also known as granulomatous slack skin, is related to skin disease and mycosis fungoides. 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, and related phenotypes are redundant skin and erythema

Wikipedia : 76 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:

59 32 (show all 10)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 redundant skin 59 32 frequent (33%) Frequent (79-30%) HP:0001582
2 erythema 59 32 frequent (33%) Frequent (79-30%) HP:0010783
3 hodgkin lymphoma 59 32 frequent (33%) Frequent (79-30%) HP:0012189
4 stiff skin 59 32 frequent (33%) Frequent (79-30%) HP:0030053
5 nephrocalcinosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0000121
6 acute kidney injury 59 32 occasional (7.5%) Occasional (29-5%) HP:0001919
7 abnormality of the lymph nodes 59 32 occasional (7.5%) Occasional (29-5%) HP:0002733
8 hypercalcemia 59 32 occasional (7.5%) Occasional (29-5%) HP:0003072
9 cutis laxa 59 Frequent (79-30%)
10 lymphoma 59 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 14)
# 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 Nitrogen Mustard Compounds Phase 2
7 Hormone Antagonists Phase 2
8 Triamcinolone diacetate Phase 2
9 Triamcinolone hexacetonide 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
14 Antineoplastic Agents, Alkylating 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:

41
Skin, T Cells, Kidney, Lymph Node

Publications for Granulomatous Slack Skin Disease

Articles related to Granulomatous Slack Skin Disease:

(show all 17)
# Title Authors Year
1
Granulomatous slack skin - seven years follow-up of a case with features of &amp;quot;parapsoriasis en plaques&amp;quot;, muscle involvement, granulomatous vasculitis, and necrobiotic changes. ( 27516031 )
2016
2
Granulomatous Lymphoproliferative Disorders: Granulomatous Slack Skin and Lymphomatoid Granulomatosis. ( 26143428 )
2015
3
Mycosis fungoides with reactive lymphoid follicles may represent an early histopathologic picture of granulomatous slack skin. ( 23521636 )
2013
4
Combined granulomatous slack skin/folliculotropic mycosis fungoides: case report. ( 22320384 )
2013
5
Granulomatous slack skin disease: a review, in comparison with mycosis fungoides. ( 22435618 )
2012
6
Foreign-body-associated granulomatous slack skin in folliculotropic mycosis fungoides of childhood. ( 19476528 )
2009
7
Granulomatous slack skin clinically and histologically masquerading as borderline leprosy in its early stages. ( 19171548 )
2009
8
Granulomatous mycosis fungoides and granulomatous slack skin: a multicenter study of the Cutaneous Lymphoma Histopathology Task Force Group of the European Organization For Research and Treatment of Cancer (EORTC). ( 19075143 )
2008
9
An unusual case of granulomatous slack skin disease with necrobiosis. ( 18806490 )
2008
10
Granulomatous slack skin with a translocation t(3;9)(q12;p24). ( 17460466 )
2007
11
Granulomatous slack skin disease in a child: the outcome. ( 18035987 )
2007
12
Granulomatous slack skin disease--disease features and response to pentostatin. ( 14531912 )
2003
13
Granulomatous slack skin or granulomatous mycosis fungoides -- a case report. Complete response to percutaneous radiation and interferon alpha. ( 11862472 )
2002
14
A case of primary cutaneous CD30+ T-cell lymphoproliferative disorder with features of granulomatous slack skin disease. ( 12410714 )
2002
15
Granulomatous slack skin: cytogenetic and molecular analyses. ( 8143283 )
1994
16
Granulomatous slack skin: a clinicopathological and immunohistochemical study of three cases. ( 1531613 )
1992
17
Lymphoproliferative nature of granulomatous slack skin: clonal rearrangement of the T-cell receptor beta-gene. ( 1316105 )
1992

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
17 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 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
Content
Loading form....