MCID: GRN032
MIFTS: 32

Granulomatous Slack Skin Disease

Categories: Blood diseases, Immune diseases, Rare diseases, Skin diseases

Aliases & Classifications for Granulomatous Slack Skin Disease

MalaCards integrated aliases for Granulomatous Slack Skin Disease:

Name: Granulomatous Slack Skin Disease 54 74
Granulomatous Slack Skin 54 60 56 74

Characteristics:

Orphanet epidemiological data:

60
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 : 54 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 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 lymph node, and related phenotypes are erythema and redundant skin

Wikipedia : 77 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:

(show all 19)
# Related Disease Score Top Affiliating Genes
1 skin disease 10.5
2 lymphoma 10.5
3 cutaneous t cell lymphoma 10.4
4 vasculitis 10.2
5 folliculotropic mycosis fungoides 10.2
6 mycosis fungoides 10.1
7 lymphoproliferative syndrome 10.1
8 leprosy 3 10.1
9 autoimmune lymphoproliferative syndrome, type v 10.1
10 lymphomatoid granulomatosis 10.1
11 inguinal hernia 10.1
12 borderline leprosy 10.1
13 ichthyosis 10.1
14 giant cell tumor 10.1
15 sezary's disease 10.1
16 parapsoriasis 10.1
17 degos 'en cocarde' erythrokeratoderma 10.1
18 hansen's disease 10.1
19 ichthyosis, acquired 10.1

Graphical network of the top 20 diseases related to Granulomatous Slack Skin Disease:



Diseases related to Granulomatous Slack Skin Disease

Symptoms & Phenotypes for Granulomatous Slack Skin Disease

Human phenotypes related to Granulomatous Slack Skin Disease:

60 33 (show all 10)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 erythema 60 33 frequent (33%) Frequent (79-30%) HP:0010783
2 redundant skin 60 33 frequent (33%) Frequent (79-30%) HP:0001582
3 hodgkin lymphoma 60 33 frequent (33%) Frequent (79-30%) HP:0012189
4 stiff skin 60 33 frequent (33%) Frequent (79-30%) HP:0030053
5 nephrocalcinosis 60 33 occasional (7.5%) Occasional (29-5%) HP:0000121
6 hypercalcemia 60 33 occasional (7.5%) Occasional (29-5%) HP:0003072
7 acute kidney injury 60 33 occasional (7.5%) Occasional (29-5%) HP:0001919
8 abnormality of the lymph nodes 60 33 occasional (7.5%) Occasional (29-5%) HP:0002733
9 lymphoma 60 Frequent (79-30%)
10 cutis laxa 60 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 15)
# 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 Antineoplastic Agents, Alkylating Phase 2
4 Triamcinolone hexacetonide Phase 2
5 Immunologic Factors Phase 2
6 Anti-Inflammatory Agents Phase 2
7 glucocorticoids Phase 2
8 Triamcinolone diacetate Phase 2
9 triamcinolone acetonide Phase 2
10 Hormone Antagonists Phase 2
11 Alkylating Agents Phase 2
12 Immunosuppressive Agents Phase 2
13 Nitrogen Mustard Compounds Phase 2
14 Hormones Phase 2
15 Hormones, Hormone Substitutes, and Hormone Antagonists 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:

42
Skin, T Cells, Lymph Node, Kidney

Publications for Granulomatous Slack Skin Disease

Articles related to Granulomatous Slack Skin Disease:

(show top 50) (show all 65)
# Title Authors Year
1
Severe hypercalcemia complicating granulomatous slack skin disease : An exceptional case. ( 31026372 )
2019
2
Granulomatous slack skin syndrome: Report of a unique case. ( 29067930 )
2018
3
Granulomatous slack skin: an unusual variant of cutaneous T-cell lymphoma. ( 26694877 )
2017
4
Granulomatous slack skin: a rare subtype of mycosis fungoides. ( 29166509 )
2017
5
Granulomatous slack skin presenting as diffuse poikiloderma and necrotic ulcers, with features of granulomatous vasculitis and response to oral prednisone, acitretin, and oral psoralen plus ultraviolet light therapy-A case report. ( 28748211 )
2017
6
Granulomatous slack skin with vascular involvement. ( 28872761 )
2017
7
Kutanes elastolytisches T-Zell-Lymphom (Granulomatous Slack Skin) mit Gefäßbeteiligung. ( 28976105 )
2017
8
Granulomatous slack skin - seven years follow-up of a case with features of "parapsoriasis en plaques", muscle involvement, granulomatous vasculitis, and necrobiotic changes. ( 27516031 )
2016
9
Granulomatous slack skin: a case report. ( 27020219 )
2016
10
A case of granulomatous slack skin cutaneous T-cell lymphoma: PET/CT imaging findings. ( 30363213 )
2015
11
Granulomatous slack skin T-cell lymphoma: an important differential diagnosis with giant cell tumor of soft tissue. ( 26734874 )
2015
12
Granulomatous Lymphoproliferative Disorders: Granulomatous Slack Skin and Lymphomatoid Granulomatosis. ( 26143428 )
2015
13
Granulomatous slack skin-like clinical findings in Sézary syndrome. ( 26265466 )
2015
14
Granulomatous mycosis fungoides with clinical features of granulomatous slack skin. ( 24738107 )
2014
15
Granulomatous slack skin with systemic involvement and a fatal outcome in an adolescent. ( 24890870 )
2014
16
Combined granulomatous slack skin/folliculotropic mycosis fungoides: case report. ( 22320384 )
2013
17
Mycosis fungoides with reactive lymphoid follicles may represent an early histopathologic picture of granulomatous slack skin. ( 23521636 )
2013
18
Granulomatous slack skin with extracutaneous involvement and a good response to bortezomib-based chemotherapy. ( 24211790 )
2013
19
Granulomatous slack skin disease: a review, in comparison with mycosis fungoides. ( 22435618 )
2012
20
Granulomatous slack skin. Histopathology diagnosis preceding clinical manifestations by 12 years. ( 23329989 )
2012
21
F-18 FDG PET/CT in granulomatous slack skin. ( 20090455 )
2010
22
Granulomatous slack skin clinically and histologically masquerading as borderline leprosy in its early stages. ( 19171548 )
2009
23
Foreign-body-associated granulomatous slack skin in folliculotropic mycosis fungoides of childhood. ( 19476528 )
2009
24
Granulomatous slack skin presenting as acquired ichthyosis and muscle masses. ( 19170408 )
2009
25
Granulomatous slack skin responds to UVA1 phototherapy. ( 19546523 )
2009
26
Granulomatous slack skin: assessment of disease progression and treatment response using positron emission tomography/computed tomography. ( 19951886 )
2009
27
An unusual case of granulomatous slack skin disease with necrobiosis. ( 18806490 )
2008
28
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
29
Granulomatous slack skin: report of a case with response to electron beam therapy. ( 17968684 )
2008
30
Granulomatous slack skin disease in a child: the outcome. ( 18035987 )
2007
31
Topical nitrogen mustard for the treatment of granulomatous slack skin. ( 17298108 )
2007
32
Granulomatous slack skin with a translocation t(3;9)(q12;p24). ( 17460466 )
2007
33
Granulomatous slack skin with clonal T-cell receptor-gamma gene rearrangement in skin and lymph node. ( 17573870 )
2007
34
Granulomatous slack skin. ( 17673389 )
2007
35
Bilateral inguinal hernia with dislocation of great saphenous vein as complication of long-standing granulomatous slack skin: a case report. ( 16684291 )
2006
36
Granulomatous slack skin. ( 17459306 )
2006
37
Granulomatous slack skin with extracutaneous involvement. ( 16172332 )
2005
38
Prednisone combined with interferon for the treatment of one case of generalized granulomatous slack skin. ( 16463692 )
2005
39
Granulomatous slack skin without evidence of a clonal T-cell proliferation. ( 14726855 )
2004
40
Granulomatous slack skin disease--disease features and response to pentostatin. ( 14531912 )
2003
41
Granulomatous slack skin: treatment with extensive surgery and review of the literature. ( 12771496 )
2003
42
A case of primary cutaneous CD30+ T-cell lymphoproliferative disorder with features of granulomatous slack skin disease. ( 12410714 )
2002
43
Granulomatous slack skin or granulomatous mycosis fungoides -- a case report. Complete response to percutaneous radiation and interferon alpha. ( 11862472 )
2002
44
Granulomatous slack skin: a distinct disorder or a variant of mycosis fungoides? ( 11411914 )
2001
45
Granulomatous slack skin: an ultrastructural study. ( 11168751 )
2001
46
Granulomatous slack skin: a case of unusual variant of mycosis fungoides. ( 11775552 )
2000
47
Granulomatous slack skin: successful treatment with recombinant interferon-gamma. ( 10730774 )
2000
48
Granulomatous slack skin. ( 10849132 )
2000
49
Granulomatous mycosis fungoides: report of a case with some histopathologic features of granulomatous slack skin. ( 10218676 )
1999
50
Granulomatous slack skin. Report of three patients with an updated review of the literature. ( 9669112 )
1998

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

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72 TGDB
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74 UMLS
75 UMLS via Orphanet
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