MCID: GRN032
MIFTS: 28

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 52 71
Granulomatous Slack Skin 52 58 54 71

Characteristics:

Orphanet epidemiological data:

58
granulomatous slack skin
Inheritance: Not applicable; Prevalence: <1/1000000 (Worldwide); Age of onset: Adult; Age of death: normal life expectancy;

Classifications:

Orphanet: 58  
Rare skin diseases
Rare haematological diseases


External Ids:

ICD10 via Orphanet 33 C84.0
UMLS via Orphanet 72 C0376407 C0457002
Orphanet 58 ORPHA33111
UMLS 71 C0376407 C0457002

Summaries for Granulomatous Slack Skin Disease

NIH Rare Diseases : 52 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). Affiliated tissues include skin, t cells and lymph node, and related phenotypes are erythema and redundant skin

Wikipedia : 74 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 31)
# Related Disease Score Top Affiliating Genes
1 skin disease 10.5
2 lymphoma 10.5
3 mycosis fungoides 10.4
4 cutis laxa 10.4
5 cutaneous t cell lymphoma 10.3
6 sarcoidosis 1 10.3
7 anaplastic large cell lymphoma 10.3
8 vasculitis 10.3
9 folliculotropic mycosis fungoides 10.3
10 peripheral t-cell lymphoma 10.1
11 lymphoproliferative syndrome 10.1
12 sezary's disease 10.1
13 lymphoma, hodgkin, classic 10.1
14 leprosy 3 10.1
15 diffuse large b-cell lymphoma 10.1
16 inguinal hernia 10.1
17 borderline leprosy 10.1
18 ichthyosis 10.1
19 basal cell carcinoma 10.1
20 histiocytosis 10.1
21 granuloma annulare 10.1
22 seminoma 10.1
23 b-cell lymphoma 10.1
24 parapsoriasis 10.1
25 chromosomal triplication 10.1
26 hansen's disease 10.1
27 ichthyosis, acquired 10.1
28 lymphomatoid granulomatosis 10.1
29 testicular seminoma 10.1
30 primary cutaneous t-cell lymphoma 10.1
31 alk-negative anaplastic large cell lymphoma 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:

58 31 (show all 10)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 erythema 58 31 frequent (33%) Frequent (79-30%) HP:0010783
2 redundant skin 58 31 frequent (33%) Frequent (79-30%) HP:0001582
3 hodgkin lymphoma 58 31 frequent (33%) Frequent (79-30%) HP:0012189
4 stiff skin 58 31 frequent (33%) Frequent (79-30%) HP:0030053
5 nephrocalcinosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000121
6 hypercalcemia 58 31 occasional (7.5%) Occasional (29-5%) HP:0003072
7 acute kidney injury 58 31 occasional (7.5%) Occasional (29-5%) HP:0001919
8 abnormality of the lymph nodes 58 31 occasional (7.5%) Occasional (29-5%) HP:0002733
9 lymphoma 58 Frequent (79-30%)
10 cutis laxa 58 Frequent (79-30%)

Drugs & Therapeutics for Granulomatous Slack Skin Disease

Search Clinical Trials , 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:

40
Skin, T Cells, Lymph Node, Kidney, Nk Cells, B Cells

Publications for Granulomatous Slack Skin Disease

Articles related to Granulomatous Slack Skin Disease:

(show top 50) (show all 90)
# Title Authors PMID Year
1
Lymphoproliferative nature of granulomatous slack skin: clonal rearrangement of the T-cell receptor beta-gene. 54 61
1316105 1992
2
Granulomatous Slack Skin: A Case Report. 61
32363106 2020
3
Granulomatous slack skin mycosis fungoides developing simultaneously with sarcoid-like lesions in a patient with repeated anabolic injections in the past? 61
31854482 2020
4
Severe hypercalcemia complicating granulomatous slack skin disease: an exceptional case. 61
31026372 2019
5
Granulomatous slack skin: case report with electron microscopic features. 61
31450274 2019
6
Granulomatous slack skin syndrome: Report of a unique case. 61
29067930 2018
7
Granulomatous slack skin with vascular involvement. 61
28872761 2017
8
Kutanes elastolytisches T-Zell-Lymphom (Granulomatous Slack Skin) mit Gefäßbeteiligung. 61
28976105 2017
9
Granulomatous slack skin: a rare subtype of mycosis fungoides. 61
29166509 2017
10
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. 61
28748211 2017
11
[Granulomatous slack skin associated with metastatic testicular seminoma]. 61
28396065 2017
12
Granulomatous slack skin: an unusual variant of cutaneous T-cell lymphoma. 61
26694877 2017
13
[Granulomatous slack skin associated with ALK-negative anaplastic large cell lymphoma]. 61
28056306 2016
14
Unusual variants of mycosis fungoides. 61
29225700 2016
15
Granulomatous slack skin - seven years follow-up of a case with features of "parapsoriasis en plaques", muscle involvement, granulomatous vasculitis, and necrobiotic changes. 61
27516031 2016
16
Granulomatous slack skin: a case report. 61
27020219 2016
17
Granulomatous slack skin-like clinical findings in Sézary syndrome. 61
26265466 2015
18
Granulomatous slack skin T-cell lymphoma: an important differential diagnosis with giant cell tumor of soft tissue. 61
26734874 2015
19
Granulomatous Lymphoproliferative Disorders: Granulomatous Slack Skin and Lymphomatoid Granulomatosis. 61
26143428 2015
20
A case of granulomatous slack skin cutaneous T-cell lymphoma: PET/CT imaging findings. 61
30363213 2015
21
Granulomatous slack skin with systemic involvement and a fatal outcome in an adolescent. 61
24890870 2014
22
Mycosis Fungoides Variants. 61
26837197 2014
23
[Granulomatous mycosis fungoides: combination therapy with bexarotene and PUVA]. 61
24671704 2014
24
Granulomatous mycosis fungoides with clinical features of granulomatous slack skin. 61
24738107 2014
25
Combined granulomatous slack skin/folliculotropic mycosis fungoides: case report. 61
22320384 2013
26
Granulomatous slack skin with extracutaneous involvement and a good response to bortezomib-based chemotherapy. 61
24211790 2013
27
Mycosis fungoides with reactive lymphoid follicles may represent an early histopathologic picture of granulomatous slack skin. 61
23521636 2013
28
Granulomatous slack skin disease: a review, in comparison with mycosis fungoides. 61
22435618 2012
29
Granulomatous slack skin. Histopathology diagnosis preceding clinical manifestations by 12 years. 61
23329989 2012
30
Unusual variants of mycosis fungoides. 61
21980706 2011
31
[Granulomatous slack skin with anaplastic large cell lymphoma: report of a case]. 61
21616005 2011
32
[Epitheliotropic lymphomas: better identification for improved treatment]. 61
22043631 2010
33
F-18 FDG PET/CT in granulomatous slack skin. 61
20090455 2010
34
Granulomatous slack skin: assessment of disease progression and treatment response using positron emission tomography/computed tomography. 61
19951886 2009
35
Cutaneous lymphomas showing prominent granulomatous component: clinicopathological features in a series of 16 cases. 61
19207677 2009
36
Foreign-body-associated granulomatous slack skin in folliculotropic mycosis fungoides of childhood. 61
19476528 2009
37
[Granulomatous slack skin: report of a case]. 61
19575903 2009
38
Granulomatous slack skin clinically and histologically masquerading as borderline leprosy in its early stages. 61
19171548 2009
39
Granulomatous slack skin responds to UVA1 phototherapy. 61
19546523 2009
40
Granulomatous slack skin presenting as acquired ichthyosis and muscle masses. 61
19170408 2009
41
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). 61
19075143 2008
42
An unusual case of granulomatous slack skin disease with necrobiosis. 61
18806490 2008
43
Granulomatous slack skin: report of a case with response to electron beam therapy. 61
17968684 2008
44
Anetodermic mycosis fungoides: a new clinicopathological variant of mycosis fungoides. 61
17999702 2008
45
Granulomatous slack skin disease in a child: the outcome. 61
18035987 2007
46
Granulomatous slack skin. 61
17673389 2007
47
Granulomatous slack skin with clonal T-cell receptor-gamma gene rearrangement in skin and lymph node. 61
17573870 2007
48
Granulomatous slack skin with a translocation t(3;9)(q12;p24). 61
17460466 2007
49
Mycosis fungoides: a dermatological masquerader. 61
17199560 2007
50
Topical nitrogen mustard for the treatment of granulomatous slack skin. 61
17298108 2007

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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11 DGIdb
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61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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