MCID: LNR006
MIFTS: 44

Linear Iga Disease

Categories: Rare diseases, Skin diseases, Immune diseases

Aliases & Classifications for Linear Iga Disease

MalaCards integrated aliases for Linear Iga Disease:

Name: Linear Iga Disease 53
Linear Iga Bullous Dermatosis 53 73
Linear Iga Dermatosis 53 59
Linear Immunoglobulin a Dermatosis 53
Lad 53

Classifications:

Orphanet: 59  
Rare skin diseases


External Ids:

Orphanet 59 ORPHA46488
UMLS via Orphanet 74 C0406650
ICD10 via Orphanet 34 L10.8
UMLS 73 C0406650

Summaries for Linear Iga Disease

MalaCards based summary : Linear Iga Disease, also known as linear iga bullous dermatosis, is related to pemphigoid gestationis and herpes gestationis. 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, neutrophil and colon, and related phenotypes are oral ulcer and epistaxis

Wikipedia : 76 Linear IgA bullous dermatosis is frequently associated with medication exposure, especially vancomycin,... 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 63)
# Related Disease Score Top Affiliating Genes
1 pemphigoid gestationis 29.5 COL17A1 DST
2 herpes gestationis 29.4 COL17A1 DST
3 lichen planus 28.9 COL17A1 DST
4 epidermolysis bullosa 28.8 COL17A1 DST ITGB4
5 pemphigus 28.1 CD79A COL17A1 DST
6 cicatricial pemphigoid 27.8 CD79A COL17A1 DST ITGB4
7 bullous pemphigoid 27.7 CD79A COL17A1 DST ITGB4
8 epidermolysis bullosa acquisita 27.3 CD79A COL17A1 DST ITGB4
9 recessive dystrophic epidermolysis bullosa-generalized other 11.4
10 dermatitis 10.2
11 dermatitis herpetiformis 10.2
12 dermatitis herpetiformis, familial 10.2
13 colitis 10.1
14 ulcerative colitis 10.1
15 gingivitis 10.1
16 neurotic excoriation 9.9 COL17A1 DST
17 epidermolysis bullosa simplex with muscular dystrophy 9.9 COL17A1 DST
18 erythema multiforme 9.9
19 thyroiditis 9.8
20 hashimoto thyroiditis 9.8
21 ecthyma 9.8 COL17A1 DST
22 kindler syndrome 9.8 DST ITGB4
23 hepatitis 9.7
24 neutropenia 9.7
25 contact dermatitis 9.7
26 prurigo nodularis 9.7
27 thalassemia 9.7
28 bronchiolitis 9.7
29 iga glomerulonephritis 9.7
30 inflammatory bowel disease 9.7
31 gestational trophoblastic neoplasm 9.7
32 lymphocytic colitis 9.7
33 lymphoproliferative syndrome 9.7
34 systemic lupus erythematosus 9.7
35 mucositis 9.7
36 hydatidiform mole, recurrent, 1 9.7
37 sensorineural hearing loss 9.7
38 herpes zoster 9.7
39 lupus erythematosus 9.7
40 autoimmune lymphoproliferative syndrome 9.7
41 hepatitis a 9.7
42 psoriasis 9.7
43 alpha-thalassemia 9.7
44 balanitis 9.7
45 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 9.7
46 crohn's disease 9.7
47 pompholyx 9.7
48 paraneoplastic pemphigus 9.7 COL17A1 DST
49 pyloric atresia 9.6 COL17A1 ITGB4 LAD1
50 lichen planus pemphigoides 9.5 COL17A1 DST ITGB4

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:

59 32 (show all 9)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 oral ulcer 59 32 frequent (33%) Frequent (79-30%) HP:0000155
2 epistaxis 59 32 occasional (7.5%) Occasional (29-5%) HP:0000421
3 pruritus 59 32 occasional (7.5%) Occasional (29-5%) HP:0000989
4 inflammation of the large intestine 59 32 occasional (7.5%) Occasional (29-5%) HP:0002037
5 autoimmunity 59 32 hallmark (90%) Very frequent (99-80%) HP:0002960
6 abnormal blistering of the skin 59 32 hallmark (90%) Very frequent (99-80%) HP:0008066
7 bladder neoplasm 59 32 occasional (7.5%) Occasional (29-5%) HP:0009725
8 renal neoplasm 59 32 occasional (7.5%) Occasional (29-5%) HP:0009726
9 papule 59 32 hallmark (90%) Very frequent (99-80%) HP:0200034

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, Nutraceutical Phase 1 58-05-9 143 6006
3
Folic Acid Approved, Nutraceutical, Vet_approved Phase 1 59-30-3 6037
4 Folic Acid Antagonists Phase 1
5 Anti-Bacterial Agents Phase 1
6 Vitamin B Complex Phase 1
7 Anti-Infective Agents Phase 1
8 Antiparasitic Agents Phase 1
9 Antiprotozoal Agents Phase 1
10 Antimalarials Phase 1
11 Folate Nutraceutical Phase 1
12 Vitamin B9 Nutraceutical 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 Register of Autoimmune Bullous Dermatoses Completed NCT02899923
3 Genetic Susceptibility and Influence of the Microbiomae in Bullous Pemphigoid Recruiting NCT02874079 Not Applicable
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:

41
Skin, Neutrophil, Colon, Placenta, T Cells

Publications for Linear Iga Disease

Articles related to Linear Iga Disease:

(show top 50) (show all 173)
# Title Authors Year
1
Linear IgA bullous dermatosis mimicking oral lichen planus. ( 28670088 )
2017
2
Intraepidermal neutrophilic dermatosis type of IgA pemphigus with circulating linear IgA disease antibodies associated with ulcerative colitis. ( 28653439 )
2017
3
Piperacillin-tazobactam-induced linear IgA bullous dermatosis presenting clinically as Stevens-Johnson syndrome/toxic epidermal necrolysis overlap. ( 28084616 )
2017
4
Linear IgA Disease in a Pregnant Woman with Autoantibodies to the Non-collagenous 16A Domain of Type XVII Collagen. ( 27786348 )
2016
5
Vancomycin-associated linear IgA disease mimicking toxic epidermal necrolysis. ( 28300888 )
2016
6
Immunohistochemical localization of basement membrane laminin 5 and collagen IV in adult linear IgA disease. ( 25771892 )
2015
7
Linear IgA disease associated with ulcerative colitis: the role of surgery. ( 24635071 )
2014
8
Drug-induced linear IgA bullous dermatosis simulating toxic epidermal necrolysis. ( 25306799 )
2014
9
Coexistence of antilaminin-332-type mucous membrane pemphigoid, lamina lucida-type linear IgA bullous dermatosis and SjAPgren syndrome. ( 23397948 )
2013
10
Fluorescence overlay antigen mapping using laser scanning confocal microscopy differentiates linear IgA bullous dermatosis from epidermolysis bullosa acquisita mediated by IgA. ( 22924407 )
2013
11
A case of linear IgA disease limited to the oral mucosa. ( 24353808 )
2013
12
Linear IgA bullous dermatosis: an unusual cause of upper eyelid cicatricial entropion. ( 23446308 )
2013
13
Linear IgA disease in an adult with unusual clinical features. ( 23741669 )
2013
14
Linear IgA bullous dermatosis with a prurigo nodularis-like appearance. ( 23306219 )
2013
15
Amlodipine-induced linear IgA disease. ( 22299719 )
2012
16
Donepezil-induced linear IgA disease. ( 21689148 )
2012
17
Linear IgA disease: clinical presentation, diagnosis, and pathogenesis. ( 22560137 )
2012
18
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
19
Tinea corporis bullosa due to Microsporum canis mimicking linear IgA bullous dermatosis. ( 23131347 )
2012
20
Management of linear IgA disease. ( 21925008 )
2011
21
Linear IgA disease: complicated by alpha thalassemia. ( 21793892 )
2011
22
Vancomycin-induced linear IgA bullous dermatosis mimicking toxic epidermal necrolysis. ( 21727724 )
2011
23
Incipiens linear IgA disease with IgA antibodies directed against 200-kDa epidermal antigens. ( 21515442 )
2011
24
Linear IgA disease limited to the oral mucosa. ( 21839337 )
2011
25
Linear IgA disease: clinical presentation, diagnosis, and pathogenesis. ( 21605811 )
2011
26
Linear IgA disease: successful application of immunoadsorption and review of the literature. ( 20130384 )
2010
27
Drug-associated linear IgA disease mimicking toxic epidermal necrolysis. ( 19077099 )
2009
28
A newborn with bullous pemphigoid associated with linear IgA bullous dermatosis. ( 19588060 )
2009
29
Childhood linear IgA bullous dermatosis in Tunisia. ( 19250401 )
2009
30
Linear IgA bullous dermatosis induced by interferon-alpha 2a. ( 19508467 )
2009
31
Linear IgA disease and desquamative gingivitis: time for inclusion in mucous membrane pemphigoid. ( 18954346 )
2008
32
Linear IgA disease of childhood developing IgA nephropathy. ( 18577038 )
2008
33
Drug induced linear IgA disease with unusual features: Koebner phenomenon, local insulin sensitivity and annular blister of the nipples. ( 19111146 )
2008
34
Dermatitis herpetiformis: potential for confusion with linear IgA bullous dermatosis on direct immunofluorescence. ( 18319038 )
2008
35
IgA autoantibodies against the NC16a domain of BP180 but not 120-kDa LAD-1 detected in a patient with linear IgA disease. ( 18363761 )
2008
36
Localized linear IgA disease associated with monoclonal gammapathy of undetermined significance. ( 17373989 )
2007
37
Dermal-binding linear IgA disease: an uncommon subset of a rare immunobullous disease. ( 17459072 )
2007
38
Subacute prurigo-like linear IgA disease. ( 17910726 )
2007
39
Localized linear IgA disease showing contact dermatitis-like eruption. ( 17540647 )
2007
40
A rare case of desquamative gingivitis due to linear IgA disease: morphological and immunofluorescence features. ( 18210762 )
2007
41
Successful treatment of oral linear IgA disease using mycophenolate. ( 17197208 )
2007
42
A case of linear IgA disease in a child with IgA and IgG circulating antibodies directed to BPAg2. ( 18173529 )
2007
43
Ocular linear IgA disease resulting in blindness. ( 16785392 )
2006
44
Linear IgA disease presenting as prurigo nodularis. ( 16882196 )
2006
45
Paraneoplastic pemphigus resembling linear IgA bullous dermatosis. ( 16961519 )
2006
46
Vancomycin-associated linear IgA bullous dermatosis mimicking toxic epidermal necrolysis. ( 16911399 )
2006
47
A case of mixed bullous disease of epidermolysis bullosa acquisita and linear IgA bullous dermatosis. ( 16088162 )
2005
48
Successful treatment of linear IgA disease with mycophenolate mofetil as a corticosteroid sparing agent. ( 15807696 )
2005
49
Coexistence of psoriasis and linear IgA disease in a patient with recent herpes zoster infection. ( 16197377 )
2005
50
A case of linear IgA disease occurring in a patient with colonic Crohn's disease. ( 16225624 )
2005

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 cell-matrix adhesion GO:0007160 9.32 COL17A1 ITGB4
2 integrin-mediated signaling pathway GO:0007229 9.26 DST ITGB4
3 response to wounding GO:0009611 9.16 DST ITGB4
4 cell motility GO:0048870 8.96 DST ITGB4
5 hemidesmosome assembly GO:0031581 8.62 COL17A1 ITGB4

Sources for Linear Iga 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
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