LAD
MCID: LNR006
MIFTS: 43

Linear Iga Disease (LAD)

Categories: Immune diseases, Rare diseases, Skin 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 paraneoplastic pemphigus and epidermolysis bullosa, junctional, non-herlitz type. 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 leucovorin and Dapsone have been mentioned in the context of this disorder. Affiliated tissues include skin, colon and neutrophil, and related phenotypes are abnormal blistering of the skin and pruritus

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 105)
# Related Disease Score Top Affiliating Genes
1 paraneoplastic pemphigus 29.9 COL17A1 DST
2 epidermolysis bullosa, junctional, non-herlitz type 29.8 COL17A1 DST ITGB4 LAD1
3 lichen planus 29.8 COL17A1 DST
4 pyloric atresia 29.8 COL17A1 ITGB4 LAD1
5 pemphigoid gestationis 29.7 COL17A1 DST
6 epidermolysis bullosa 29.7 COL17A1 DST ITGB4
7 herpes gestationis 29.7 COL17A1 DST
8 ocular cicatricial pemphigoid 29.7 CD79A ITGB4
9 junctional epidermolysis bullosa 29.7 COL17A1 DST ITGB4
10 cicatricial pemphigoid 29.5 CD79A COL17A1 DST ITGB4
11 epidermolysis bullosa acquisita 29.4 COL17A1 DST ITGB4
12 pemphigus 29.3 CD79A COL17A1 DST
13 bullous pemphigoid 29.1 CD79A COL17A1 DST ITGB4 LAD1
14 recessive dystrophic epidermolysis bullosa-generalized other 11.5
15 renal cell carcinoma, nonpapillary 10.3
16 dermatitis 10.3
17 dermatitis herpetiformis, familial 10.3
18 dermatitis herpetiformis 10.3
19 severe cutaneous adverse reaction 10.3
20 lymphocytic leukemia 10.3
21 sclerosing cholangitis 10.3
22 cholangitis 10.3
23 x-linked intellectual disability-macrocephaly-macroorchidism syndrome 10.3
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 multiple sclerosis 10.1
30 leukemia, chronic lymphocytic 10.1
31 pemphigus vulgaris, familial 10.1
32 rheumatoid arthritis 10.1
33 pancreatic cancer 10.1
34 cholangitis, primary sclerosing 10.1
35 acute lymphocytic leukemia 10.1
36 alopecia 10.1
37 arthritis 10.1
38 hemolytic anemia 10.1
39 intermittent claudication 10.1
40 pemphigus vulgaris 10.1
41 lymphoma 10.1
42 dermatomyositis 10.1
43 allergic hypersensitivity disease 10.1
44 tinea corporis 10.1
45 entropion 10.1
46 cicatricial entropion 10.1
47 lateral sclerosis 10.1
48 adenocarcinoma 10.1
49 proctitis 10.1
50 conjunctivitis 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:

59 32 (show all 9)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 abnormal blistering of the skin 59 32 hallmark (90%) Very frequent (99-80%) HP:0008066
2 pruritus 59 32 occasional (7.5%) Occasional (29-5%) HP:0000989
3 autoimmunity 59 32 hallmark (90%) Very frequent (99-80%) HP:0002960
4 inflammation of the large intestine 59 32 occasional (7.5%) Occasional (29-5%) HP:0002037
5 epistaxis 59 32 occasional (7.5%) Occasional (29-5%) HP:0000421
6 papule 59 32 hallmark (90%) Very frequent (99-80%) HP:0200034
7 renal neoplasm 59 32 occasional (7.5%) Occasional (29-5%) HP:0009726
8 oral ulcer 59 32 frequent (33%) Frequent (79-30%) HP:0000155
9 bladder neoplasm 59 32 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
leucovorin Approved Phase 1 58-05-9 6006 143
2
Dapsone Approved, Investigational Phase 1 80-08-0 2955
3
Folic Acid Approved, Nutraceutical, Vet_approved Phase 1 59-30-3 6037
4 Anti-Infective Agents Phase 1
5 Vitamin B9 Phase 1
6 Vitamin B Complex Phase 1
7 Antiparasitic Agents Phase 1
8 Antiprotozoal Agents Phase 1
9 Folic Acid Antagonists Phase 1
10 Anti-Bacterial Agents Phase 1
11 Antimalarials Phase 1
12 Folate 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:

41
Skin, Colon, Neutrophil, Testes, T Cells, Thyroid, B Cells

Publications for Linear Iga Disease

Articles related to Linear Iga Disease:

(show top 50) (show all 342)
# Title Authors Year
1
Annular paraneoplastic bullous pemphigoid mimicking linear IgA bullous dermatosis in a 40-year-old patient. ( 29578309 )
2018
2
Linear IgA Bullous Dermatosis Preceding the Diagnosis of Primary Sclerosing Cholangitis and Ulcerative Colitis: A Case Report. ( 30461424 )
2018
3
Linear IgA bullous dermatosis associated with amyotrophic lateral sclerosis. ( 29023948 )
2018
4
Linear IgA bullous dermatosis associated with ulcerative proctitis: treatment challenge. ( 30261573 )
2018
5
Linear IgA bullous dermatosis: A rare manifestation of vancomycin hypersensitivity. ( 29128161 )
2018
6
Vancomycin Mediates IgA Autoreactivity in Drug-Induced Linear IgA Bullous Dermatosis. ( 29410066 )
2018
7
Uncomplicated Neonatal Linear IgA Bullous Dermatosis: A Case Report. ( 29502441 )
2018
8
Linear IgA bullous dermatosis induced by diclofenac sodium. ( 29667608 )
2018
9
Drug-induced linear IgA bullous dermatosis in a patient with a vancomycin-impregnated cement spacer. ( 29763476 )
2018
10
Adult linear IgA bullous dermatosis: report of three cases. ( 29924252 )
2018
11
Linear IgA bullous dermatosis presenting as chronic prurigo: unveiling of the diagnosis by serrated pattern analysis. ( 30230533 )
2018
12
Drug-induced linear IgA bullous dermatosis: a French retrospective pharmacovigilance study of 69 cases. ( 30511379 )
2018
13
Linear IgA bullous dermatosis mimicking oral lichen planus. ( 28670088 )
2017
14
Intraepidermal neutrophilic dermatosis type of IgA pemphigus with circulating linear IgA disease antibodies associated with ulcerative colitis. ( 28653439 )
2017
15
Piperacillin-tazobactam-induced linear IgA bullous dermatosis presenting clinically as Stevens-Johnson syndrome/toxic epidermal necrolysis overlap. ( 28084616 )
2017
16
Linear IgA Bullous Dermatosis Associated with Immunoglobulin Light-chain Amyloidosis. ( 27882389 )
2017
17
A clinical and serological study of linear IgA bullous dermatosis without linear immunoglobulin deposition other than IgA at the basement membrane zone using direct immunofluorescence. ( 27943257 )
2017
18
Idiopathic linear IgA bullous dermatosis: prognostic factors based on a case series of 72 adults. ( 27995619 )
2017
19
IgA tracheobronchial deposits underlie respiratory compromise in neonatal linear IgA bullous dermatosis. ( 28079922 )
2017
20
Linear IgA bullous dermatosis in a two-year-old child: possible association with aspirin. ( 28698166 )
2017
21
Prognostic factors of patients with linear IgA bullous dermatosis. ( 28731254 )
2017
22
Linear IgA Bullous Dermatosis Presenting as Toxic Epidermal Necrolysis. ( 28751000 )
2017
23
Sublamina densa-type linear IgA bullous dermatosis with IgA autoantibodies specific for type VII collagen: a case report and clinicopathological review of 32 cases. ( 29447635 )
2017
24
Flame figures in linear IgA bullous dermatosis: a novel histopathologic finding. ( 29447641 )
2017
25
A toxic epidermal necrolysis-like presentation of linear IgA bullous dermatosis treated with dapsone. ( 29469740 )
2017
26
Linear IgA Disease in a Pregnant Woman with Autoantibodies to the Non-collagenous 16A Domain of Type XVII Collagen. ( 27786348 )
2016
27
Vancomycin-associated linear IgA disease mimicking toxic epidermal necrolysis. ( 28300888 )
2016
28
Ketoprofen-induced lamina lucida-type linear IgA bullous dermatosis. ( 25286902 )
2016
29
Causative drug detection by drug-induced lymphocyte stimulation test in drug-induced linear IgA bullous dermatosis. ( 26265104 )
2016
30
False-negative direct immunofluorescence testing in vancomycin-induced linear IgA bullous dermatosis: a diagnostic pitfall. ( 27354289 )
2016
31
Vancomycin-induced linear IgA bullous dermatosis: associations. ( 27617457 )
2016
32
Linear IgA bullous dermatosis due to vancomycin and cutaneous necrosis due to terlipressin in the same patient. ( 27643543 )
2016
33
Childhood linear IgA bullous dermatosis successfully treated with oral nicotinamide. ( 27663162 )
2016
34
A Case of Linear IgA Bullous Dermatosis Associated with Systemic Lupus Erythematosus. ( 27746656 )
2016
35
Immunohistochemical localization of basement membrane laminin 5 and collagen IV in adult linear IgA disease. ( 25771892 )
2015
36
Diaminodiphenyl Sulfone-Induced Hemolytic Anemia and Alopecia in a Case of Linear IgA Bullous Dermatosis. ( 26351428 )
2015
37
Type VII collagen is the major autoantigen for sublamina densa-type linear IgA bullous dermatosis. ( 25207819 )
2015
38
Linear IgA bullous dermatosis: a retrospective study of 23 patients in Denmark. ( 25350667 )
2015
39
A case of neonatal linear IgA bullous dermatosis with severe eye involvement. ( 25708647 )
2015
40
Linear IgA Bullous Dermatosis Secondary to Infliximab Therapy in a Patient with Ulcerative Colitis. ( 26088700 )
2015
41
Vancomycin-induced linear IgA bullous dermatosis demonstrating the isomorphic phenomenon. ( 26227580 )
2015
42
Linear IgA Bullous Dermatosis: A Rare Clinicopathologic Entity with an Unusual Presentation. ( 26557220 )
2015
43
Linear IgA bullous dermatosis associated with metastatic renal cell carcinoma. ( 27051694 )
2015
44
Urticarial linear IgA bullous dermatosis (LABD) as a presenting sign of chronic lymphocytic leukemia (CLL). ( 27051795 )
2015
45
Linear IgA disease associated with ulcerative colitis: the role of surgery. ( 24635071 )
2014
46
Drug-induced linear IgA bullous dermatosis simulating toxic epidermal necrolysis. ( 25306799 )
2014
47
Linear IgA bullous dermatosis induced by moxifloxacin. ( 23731487 )
2014
48
A case of vancomycin-induced linear IgA bullous dermatosis with circulating IgA antibodies to the NC16a domain of BP180. ( 23829415 )
2014
49
A case of ultraviolet B-induced linear IgA bullous dermatosis. ( 24448131 )
2014
50
A case of vancomycin-associated linear IgA bullous dermatosis and IgA antibodies to the α3 subunit of laminin-332. ( 24641255 )
2014

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 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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