MCID: LNR006
MIFTS: 45

Linear Iga Disease

Categories: Immune diseases, Rare diseases, Skin diseases

Aliases & Classifications for Linear Iga Disease

MalaCards integrated aliases for Linear Iga Disease:

Name: Linear Iga Disease 54
Linear Iga Dermatosis 54 60 17
Linear Iga Bullous Dermatosis 54 74
Linear Immunoglobulin a Dermatosis 54
Lad 54

Classifications:

Orphanet: 60  
Rare skin diseases


External Ids:

ICD10 via Orphanet 35 L10.8
UMLS via Orphanet 75 C0406650
Orphanet 60 ORPHA46488
UMLS 74 C0406650

Summaries for Linear Iga Disease

MalaCards based summary : Linear Iga Disease, also known as linear iga dermatosis, is related to pemphigoid gestationis and paraneoplastic pemphigus. An important gene associated with Linear Iga Disease is COL17A1 (Collagen Type XVII Alpha 1 Chain), and among its related pathways/superpathways are Phospholipase-C Pathway and Collagen chain trimerization. The drugs Dapsone and leucovorin have been mentioned in the context of this disorder. Affiliated tissues include skin, thyroid and bone, and related phenotypes are abnormal blistering of the skin and autoimmunity

Wikipedia : 77 Linear IgA bullous dermatosis is a rare immune-mediated blistering skin disease frequently associated... more...

Related Diseases for Linear Iga Disease

Diseases related to Linear Iga Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 105)
# Related Disease Score Top Affiliating Genes
1 pemphigoid gestationis 29.9 COL17A1 DST
2 paraneoplastic pemphigus 29.8 COL17A1 DST
3 lichen planus 29.8 COL17A1 DST
4 pyloric atresia 29.7 COL17A1 ITGB4 LAD1
5 epidermolysis bullosa 29.6 COL17A1 DST ITGB4
6 epidermolysis bullosa, junctional, non-herlitz type 29.6 COL17A1 DST ITGB4 LAD1
7 ocular cicatricial pemphigoid 29.5 CD79A ITGB4
8 junctional epidermolysis bullosa 29.5 COL17A1 DST ITGB4
9 epidermolysis bullosa acquisita 29.3 COL17A1 DST ITGB4
10 cicatricial pemphigoid 29.2 CD79A COL17A1 DST ITGB4
11 pemphigus 29.0 CD79A COL17A1 DST
12 bullous pemphigoid 28.6 CD79A COL17A1 DST ITGB4 LAD1
13 recessive dystrophic epidermolysis bullosa-generalized other 11.6
14 dermatitis 10.3
15 dermatitis herpetiformis, familial 10.3
16 dermatitis herpetiformis 10.3
17 severe cutaneous adverse reaction 10.3
18 leukemia 10.3
19 lymphocytic leukemia 10.3
20 sclerosing cholangitis 10.3
21 cholangitis 10.3
22 x-linked intellectual disability-macrocephaly-macroorchidism syndrome 10.3
23 lymphoma 10.2
24 colitis 10.2
25 ulcerative colitis 10.2
26 gingivitis 10.2
27 amyotrophic lateral sclerosis 1 10.1
28 leukemia, chronic lymphocytic 2 10.1
29 leukemia, chronic lymphocytic 10.1
30 pemphigus vulgaris, familial 10.1
31 pancreatic cancer 10.1
32 sjogren syndrome 10.1
33 cholangitis, primary sclerosing 10.1
34 acute lymphocytic leukemia 10.1
35 alopecia 10.1
36 hemolytic anemia 10.1
37 intermittent claudication 10.1
38 pemphigus vulgaris 10.1
39 allergic hypersensitivity disease 10.1
40 tinea corporis 10.1
41 entropion 10.1
42 cicatricial entropion 10.1
43 lateral sclerosis 10.1
44 proctitis 10.1
45 conjunctivitis 10.1
46 dermatophytosis 10.1
47 amyloidosis 10.1
48 leukemia, b-cell, chronic 10.1
49 oral lichen planus 10.1
50 stevens-johnson syndrome/toxic epidermal necrolysis 10.1

Graphical network of the top 20 diseases related to Linear Iga Disease:



Diseases related to Linear Iga Disease

Symptoms & Phenotypes for Linear Iga Disease

Human phenotypes related to Linear Iga Disease:

60 33 (show all 9)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 abnormal blistering of the skin 60 33 hallmark (90%) Very frequent (99-80%) HP:0008066
2 autoimmunity 60 33 hallmark (90%) Very frequent (99-80%) HP:0002960
3 papule 60 33 hallmark (90%) Very frequent (99-80%) HP:0200034
4 oral ulcer 60 33 frequent (33%) Frequent (79-30%) HP:0000155
5 pruritus 60 33 occasional (7.5%) Occasional (29-5%) HP:0000989
6 inflammation of the large intestine 60 33 occasional (7.5%) Occasional (29-5%) HP:0002037
7 epistaxis 60 33 occasional (7.5%) Occasional (29-5%) HP:0000421
8 renal neoplasm 60 33 occasional (7.5%) Occasional (29-5%) HP:0009726
9 bladder neoplasm 60 33 occasional (7.5%) Occasional (29-5%) HP:0009725

Drugs & Therapeutics for Linear Iga Disease

Drugs for Linear Iga Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 12)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dapsone Approved, Investigational Phase 1 80-08-0 2955
2
leucovorin Approved Phase 1 58-05-9 143 6006
3
Folic Acid Approved, Nutraceutical, Vet_approved Phase 1 59-30-3 6037
4 Vitamin B9 Phase 1
5 Vitamin B Complex Phase 1
6 Antiparasitic Agents Phase 1
7 Antiprotozoal Agents Phase 1
8 Anti-Infective Agents Phase 1
9 Folic Acid Antagonists Phase 1
10 Folate Phase 1
11 Anti-Bacterial Agents Phase 1
12 Antimalarials Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Pharmacokinetics and Distribution of Dapsone in Leucocytes After Single-dose and Multiple-dose Administration Completed NCT02493283 Phase 1 Dapsone single dose;Dapsone multiple dose
2 Genetic Susceptibility and Influence of the Microbiomae in Bullous Pemphigoid Unknown status NCT02874079 Not Applicable
3 Register of Autoimmune Bullous Dermatoses Completed NCT02899923
4 Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin Diseases Recruiting NCT01952275

Search NIH Clinical Center for Linear Iga Disease

Genetic Tests for Linear Iga Disease

Anatomical Context for Linear Iga Disease

MalaCards organs/tissues related to Linear Iga Disease:

42
Skin, Thyroid, Bone, Neutrophil, Colon, Bone Marrow, Placenta

Publications for Linear Iga Disease

Articles related to Linear Iga Disease:

(show top 50) (show all 204)
# Title Authors Year
1
Different presentations of linear IgA disease in a father and daughter. ( 30803057 )
2019
2
Cephalosporin-induced linear IgA dermatosis in a child: case report and literature review. ( 30977941 )
2019
3
Intraepidermal neutrophilic dermatosis type of IgA pemphigus with circulating linear IgA disease antibodies associated with ulcerative colitis. ( 28653439 )
2018
4
Linear IgA dermatosis: An atypical manifestation of a rare disease. ( 29274682 )
2018
5
Previously misdiagnosed linear IgA dermatosis resolved with dapsone. ( 29695389 )
2018
6
Linear IgA dermatosis limited to the mucosal membranes as a rare cause of dysphagia. ( 28489233 )
2017
7
C-Terminal Processing of Collagen XVII Induces Neoepitopes for Linear IgA Dermatosis Autoantibodies. ( 28842325 )
2017
8
Eosinophilic pneumonia as complication of linear IgA dermatosis treated with dapsone. ( 27886442 )
2016
9
Vancomycin-associated linear IgA disease mimicking toxic epidermal necrolysis. ( 28300888 )
2016
10
Linear IgA dermatosis after infliximab infusion for ulcerative colitis. ( 27981215 )
2016
11
A case of linear IgA dermatosis successfully treated with tetracycline and niacinamide. ( 26694098 )
2016
12
Immunohistochemical localization of basement membrane laminin 5 and collagen IV in adult linear IgA disease. ( 25771892 )
2015
13
Mucous membrane pemphigoid with IgG autoantibodies to the 120-kDa ectodomain of type XVII collagen (BP180/linear IgA dermatosis antigen) in a patient with idiopathic thrombocytopenic purpura. ( 25178495 )
2015
14
Linear IgA dermatosis adult variant with oral manifestation: A rare case report. ( 26097313 )
2015
15
Generalized linear IgA dermatosis with palmar involvement. ( 26437279 )
2015
16
Linear IgA disease associated with ulcerative colitis: the role of surgery. ( 24635071 )
2014
17
Overlap of IgA pemphigus and linear IgA dermatosis in a patient with ulcerative colitis: a mere coincidence? ( 23824334 )
2014
18
Dapsone-induced erythema multiforme with neutropenia in a patient with linear IgA dermatosis: case report and review of the literature. ( 23829595 )
2013
19
Linear IgA disease in an adult with unusual clinical features. ( 23741669 )
2013
20
A case of linear IgA disease limited to the oral mucosa. ( 24353808 )
2013
21
Linear IgA dermatosis in a patient with primary Sjogren's syndrome. ( 22763990 )
2013
22
Linear IgA dermatosis associated with ulcerative colitis: complete and sustained remission after total colectomy. ( 24068132 )
2013
23
A child with bullous skin lesions. Linear IgA dermatosis. ( 24100394 )
2013
24
Donepezil-induced linear IgA disease. ( 21689148 )
2012
25
Amlodipine-induced linear IgA disease. ( 22299719 )
2012
26
Linear IgA disease: clinical presentation, diagnosis, and pathogenesis. ( 22560137 )
2012
27
Association between the subepidermal autoimmune blistering diseases linear IgA disease and the pemphigoid group and inflammatory bowel disease: two case reports and literature review. ( 22712854 )
2012
28
Linear IgA disease: complicated by alpha thalassemia. ( 21793892 )
2011
29
Incipiens linear IgA disease with IgA antibodies directed against 200-kDa epidermal antigens. ( 21515442 )
2011
30
Linear IgA disease: clinical presentation, diagnosis, and pathogenesis. ( 21605811 )
2011
31
Linear IgA disease limited to the oral mucosa. ( 21839337 )
2011
32
Management of linear IgA disease. ( 21925008 )
2011
33
A morbilliform variant of linear IgA dermatosis: a new case. ( 22098000 )
2011
34
Unusual clinicopathological and immunological presentation of chronic bullous dermatosis of childhood (linear IgA dermatosis). ( 22121282 )
2011
35
Linear IgA disease: successful application of immunoadsorption and review of the literature. ( 20130384 )
2010
36
What is your diagnosis? Linear IgA dermatosis. ( 21049726 )
2010
37
Ocular involvement and blindness secondary to linear IgA dermatosis. ( 21234343 )
2010
38
Atypical linear IgA dermatosis revealing angioimmunoblastic T-cell lymphoma. ( 19289783 )
2009
39
Drug-associated linear IgA disease mimicking toxic epidermal necrolysis. ( 19077099 )
2009
40
Plasmin plays a role in the in vitro generation of the linear IgA dermatosis antigen LADB97. ( 19158842 )
2009
41
Linear IgA disease and desquamative gingivitis: time for inclusion in mucous membrane pemphigoid. ( 18954346 )
2008
42
Linear IgA dermatosis after herpes zoster; Wolf's isotopic response. ( 18986367 )
2008
43
IgA autoantibodies against the NC16a domain of BP180 but not 120-kDa LAD-1 detected in a patient with linear IgA disease. ( 18363761 )
2008
44
Linear IgA disease of childhood developing IgA nephropathy. ( 18577038 )
2008
45
Drug induced linear IgA disease with unusual features: Koebner phenomenon, local insulin sensitivity and annular blister of the nipples. ( 19111146 )
2008
46
Linear IgA dermatosis: report of an infantile case and analysis of 213 cases in Japan. ( 19120770 )
2008
47
Localized linear IgA disease showing contact dermatitis-like eruption. ( 17540647 )
2007
48
A rare case of desquamative gingivitis due to linear IgA disease: morphological and immunofluorescence features. ( 18210762 )
2007
49
Localized linear IgA dermatosis induced by UV light-treatment for herpes zoster. ( 17472682 )
2007
50
Successful treatment of oral linear IgA disease using mycophenolate. ( 17197208 )
2007

Variations for Linear Iga Disease

Expression for Linear Iga Disease

Search GEO for disease gene expression data for Linear Iga Disease.

Pathways for Linear Iga Disease

GO Terms for Linear Iga Disease

Cellular components related to Linear Iga Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell junction GO:0030054 9.43 COL17A1 DST ITGB4
2 cell leading edge GO:0031252 9.16 DST ITGB4
3 basement membrane GO:0005604 9.13 COL17A1 DST LAD1
4 hemidesmosome GO:0030056 8.8 COL17A1 DST ITGB4

Biological processes related to Linear Iga Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular matrix organization GO:0030198 9.37 COL17A1 ITGB4
2 cell-matrix adhesion GO:0007160 9.32 COL17A1 ITGB4
3 integrin-mediated signaling pathway GO:0007229 9.26 DST ITGB4
4 response to wounding GO:0009611 9.16 DST ITGB4
5 cell motility GO:0048870 8.96 DST ITGB4
6 hemidesmosome assembly GO:0031581 8.8 COL17A1 DST ITGB4

Molecular functions related to Linear Iga Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 integrin binding GO:0005178 8.62 DST ITGB4

Sources for Linear Iga Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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