LAD
MCID: LNR006
MIFTS: 39

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 20
Linear Iga Dermatosis 20 58 17
Linear Iga Bullous Dermatosis 20 70
Linear Immunoglobulin a Dermatosis 20
Lad 20

Classifications:

Orphanet: 58  
Rare skin diseases


External Ids:

ICD10 via Orphanet 33 L10.8
UMLS via Orphanet 71 C0406650
Orphanet 58 ORPHA46488
UMLS 70 C0406650

Summaries for Linear Iga Disease

MalaCards based summary : Linear Iga Disease, also known as linear iga dermatosis, is related to pemphigoid and pemphigoid 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 Levoleucovorin and Dapsone have been mentioned in the context of this disorder. Affiliated tissues include skin, neutrophil and bone marrow, and related phenotypes are autoimmunity and papule

Wikipedia : 73 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 154)
# Related Disease Score Top Affiliating Genes
1 pemphigoid 30.5 ITGB4 DST COL17A1
2 pemphigoid gestationis 30.1 DST COL17A1
3 paraneoplastic pemphigus 30.1 DST COL17A1
4 bullous pemphigoid 30.0 LAD1 ITGB4 DST COL17A1
5 pemphigus vulgaris, familial 29.9 DST COL17A1
6 ocular cicatricial pemphigoid 29.9 ITGB4 CD79A
7 lichen planus 29.8 DST COL17A1
8 bullous skin disease 29.8 DST COL17A1
9 pompholyx 29.8 DST COL17A1
10 pyloric atresia 29.6 LAD1 ITGB4 COL17A1
11 epidermolysis bullosa 29.6 LAD1 ITGB4 DST COL17A1
12 epidermolysis bullosa acquisita 29.6 LAD1 ITGB4 DST COL17A1
13 epidermolysis bullosa, junctional, non-herlitz type 29.6 LAD1 ITGB4 DST COL17A1
14 pemphigus 29.3 DST COL17A1 CD79A
15 junctional epidermolysis bullosa 29.1 LAD1 ITGB4 DST COL17A1
16 cicatricial pemphigoid 29.0 LAD1 ITGB4 DST COL17A1 CD79A
17 skin disease 28.8 LAD1 ITGB4 DST COL17A1 CD79A
18 recessive dystrophic epidermolysis bullosa-generalized other 11.4
19 dermatitis herpetiformis, familial 10.5
20 dermatitis herpetiformis 10.5
21 renal cell carcinoma, nonpapillary 10.4
22 severe cutaneous adverse reaction 10.4
23 lupus erythematosus 10.4
24 erythema multiforme major 10.4
25 x-linked intellectual disability-macrocephaly-macroorchidism syndrome 10.4
26 colitis 10.3
27 dermatitis 10.3
28 gingivitis 10.3
29 ulcerative colitis 10.3
30 sclerosing cholangitis 10.3
31 cholangitis 10.3
32 alopecia 10.3
33 angioimmunoblastic t-cell lymphoma 10.2
34 b-cell lymphoma 10.2
35 amyotrophic lateral sclerosis 1 10.2
36 kaposi sarcoma 10.2
37 leukemia, chronic lymphocytic 10.2
38 vater/vacterl association 10.2
39 hepatitis c virus 10.2
40 kawasaki disease 10.2
41 diabetes mellitus, ketosis-prone 10.2
42 leukemia, acute lymphoblastic 10.2
43 cholangitis, primary sclerosing 10.2
44 deficiency anemia 10.2
45 cholelithiasis 10.2
46 dermatomyositis 10.2
47 common cold 10.2
48 trachoma 10.2
49 iron deficiency anemia 10.2
50 allergic disease 10.2

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:

58 31 (show all 9)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 autoimmunity 58 31 hallmark (90%) Very frequent (99-80%) HP:0002960
2 papule 58 31 hallmark (90%) Very frequent (99-80%) HP:0200034
3 abnormal blistering of the skin 58 31 hallmark (90%) Very frequent (99-80%) HP:0008066
4 oral ulcer 58 31 frequent (33%) Frequent (79-30%) HP:0000155
5 inflammation of the large intestine 58 31 occasional (7.5%) Occasional (29-5%) HP:0002037
6 epistaxis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000421
7 pruritus 58 31 occasional (7.5%) Occasional (29-5%) HP:0000989
8 renal neoplasm 58 31 occasional (7.5%) Occasional (29-5%) HP:0009726
9 bladder neoplasm 58 31 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
Levoleucovorin Approved, Investigational Phase 1 68538-85-2 149436
2
Dapsone Approved, Investigational Phase 1 80-08-0 2955
3
Folic acid Approved, Nutraceutical, Vet_approved Phase 1 59-30-3 6037
4 Folic Acid Antagonists Phase 1
5 Vitamin B9 Phase 1
6 Folate Phase 1
7 Antiparasitic Agents Phase 1
8 Vitamin B Complex Phase 1
9 Anti-Bacterial Agents Phase 1
10 Antimalarials Phase 1
11 Antiprotozoal Agents Phase 1
12 Anti-Infective Agents Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Pharmacokinetics and Distribution of Dapsone (DDS) in Leucocytes After Single-dose and Multiple-dose Administration in Healthy Subjects Genotyped for CYP2C9 and NAT2 and in Patients With Autoimmune Bullous Dermatoses Completed NCT02493283 Phase 1 Dapsone single dose;Dapsone multiple dose
2 Assessment of the Enrichment of Rare Coding Genetic Variants in Patients Affected by Neutrophil-Mediated Inflammatory Dermatoses Unknown status 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:

40
Skin, Neutrophil, Bone Marrow, Placenta, T Cells, Heart, Thyroid

Publications for Linear Iga Disease

Articles related to Linear Iga Disease:

(show top 50) (show all 374)
# Title Authors PMID Year
1
Incidence of pemphigoid diseases in Northern Germany in 2016 - first data from the Schleswig-Holstein Registry of Autoimmune Bullous Diseases. 61
33428263 2021
2
Prevalence and age distribution of pemphigus and pemphigoid diseases among paediatric patients in Germany. 61
32289873 2020
3
Intravenous Immunoglobulins in a Series of 32 Rare and Recalcitrant Immune Dermatoses. 61
33047147 2020
4
Linear IgA dermatosis in association with angioimmunoblastic T-cell lymphoma infiltrating the skin: A case report with literature review. 61
31482600 2020
5
Bullous lesions in a young adolescent postrenal transplant. 61
32394935 2020
6
Linear IgA dermatosis of the childhood-Report of an amoxicillin-induced case. 61
31750978 2020
7
Norfloxacin-Induced Linear IgA Dermatosis. 61
33397569 2020
8
Dyshidrosiform Bullous Pemphigoid: Case Reports and Review. 61
32064205 2020
9
Lichen Planus Pemphigoides Mimicking Toxic Epidermal Necrolysis. 61
31145106 2019
10
Linear IgA Disease of the Gingiva Following Nivolumab Therapy. 61
31246639 2019
11
Mucous membrane pemphigoid and oral blistering diseases. 61
31102296 2019
12
Review of autoimmune blistering diseases: the Pemphigoid diseases. 61
31087464 2019
13
Alopecia in Autoimmune Blistering Diseases: A Systematic Review of Pathogenesis and Clinical Features of Disease. 61
31559249 2019
14
Different presentations of linear IgA disease in a father and daughter. 61
30803057 2019
15
Cephalosporin-induced linear IgA dermatosis in a child: Case report and literature review. 61
30977941 2019
16
Linear IgA Disease: A Rare Complication of Vancomycin. 61
31410331 2019
17
Oral autoimmune vesicobullous diseases: Classification, clinical presentations, molecular mechanisms, diagnostic algorithms, and management. 61
31090146 2019
18
Drug-induced Linear IgA Bullous Dermatosis: A Case Report and Review of the Literature. 61
30809685 2019
19
["Decoratively figured blisters" on the whole integument in initially diagnosed ulcerative colitis]. 61
30460586 2019
20
Lichen Planus Pemphigoides: From Lichenoid Inflammation to Autoantibody-Mediated Blistering. 61
31312198 2019
21
Complement Activation in Autoimmune Bullous Dermatoses: A Comprehensive Review. 61
31293600 2019
22
Linear IgA dermatosis: An atypical manifestation of a rare disease. 61
29274682 2018
23
Previously misdiagnosed linear IgA dermatosis resolved with dapsone. 61
29695389 2018
24
Linear immunoglobulin A dermatosis mimicking toxic epidermal necrolysis: a case report of etanercept treatment. 61
28222222 2018
25
Blistering diseases in the mature patient. 61
29566927 2018
26
The Value of Direct Immunofluorescence on Proteinase-Digested Formalin-Fixed Paraffin-Embedded Skin Biopsies. 61
28816740 2018
27
Effectiveness and Safety of Rituximab in Recalcitrant Pemphigoid Diseases. 61
29520266 2018
28
Intraepidermal neutrophilic dermatosis type of IgA pemphigus with circulating linear IgA disease antibodies associated with ulcerative colitis. 61
28653439 2018
29
C-Terminal Processing of Collagen XVII Induces Neoepitopes for Linear IgA Dermatosis Autoantibodies. 61
28842325 2017
30
Linear immunoglobulin A/G bullous dermatosis associated with ulcerative colitis. 61
28623863 2017
31
Spectrum of Autoimmune Bullous Diseases in Northern Greece. A 4-year Retrospective Study and Review of the Literature. 61
29252171 2017
32
Linear IgA dermatosis limited to the mucosal membranes as a rare cause of dysphagia. 61
28489233 2017
33
Paraneoplastic Pemphigus and Autoimmune Blistering Diseases Associated with Neoplasm: Characteristics, Diagnosis, Associated Neoplasms, Proposed Pathogenesis, Treatment. 61
27878477 2017
34
Eosinophilic pneumonia as complication of linear IgA dermatosis treated with dapsone. 61
27886442 2016
35
Combined Use of Rituximab and Intravenous Immunoglobulin for Severe Autoimmune Cicatricial Conjunctivitis-An Interventional Case Series. 61
27661067 2016
36
Linear IgA dermatosis after infliximab infusion for ulcerative colitis. 61
27981215 2016
37
A Comparative Analysis Between Antibiotic- and Nonantibiotic-Associated Delayed Cutaneous Adverse Drug Reactions. 61
27283055 2016
38
Incidence of autoimmune bullous diseases in Serbia: a 20-year retrospective study. 61
27767273 2016
39
Bullous Pemphigoid with Lymphocytic Colitis: A Case Report and Short Literature Review. 61
27469343 2016
40
Vancomycin-associated linear IgA disease mimicking toxic epidermal necrolysis. 61
28300888 2016
41
A case of linear IgA dermatosis successfully treated with tetracycline and niacinamide. 61
26694098 2016
42
The "Pearls" of Multidisciplinary Team: Conquering the Uncommon Rosette Rash. 61
28070436 2016
43
Reliability of the autoimmune bullous disease quality of life (ABQOL) questionnaire in the USA. 61
25795375 2015
44
Generalized linear IgA dermatosis with palmar involvement. 61
26437279 2015
45
Immunohistochemical localization of basement membrane laminin 5 and collagen IV in adult linear IgA disease. 61
25771892 2015
46
Concurrent Drug-Induced Linear Immunoglobulin A Dermatosis and Immunoglobulin A Nephropathy. 61
26082590 2015
47
Long-Term Balancing Selection in LAD1 Maintains a Missense Trans-Species Polymorphism in Humans, Chimpanzees, and Bonobos. 61
25605789 2015
48
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. 61
25178495 2015
49
Immune-mediated diseases: what can be found in the oral cavity? 61
25514833 2015
50
Linear IgA dermatosis adult variant with oral manifestation: A rare case report. 61
26097313 2015

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 9.5 ITGB4 DST COL17A1
2 cell leading edge GO:0031252 9.26 ITGB4 DST
3 basement membrane GO:0005604 9.26 LAD1 ITGB4 DST COL17A1
4 basal plasma membrane GO:0009925 9.16 ITGB4 DST
5 hemidesmosome GO:0030056 8.8 ITGB4 DST COL17A1

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

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

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

# Name GO ID Score Top Affiliating Genes
1 structural molecule activity GO:0005198 8.96 LAD1 DST
2 integrin binding GO:0005178 8.62 ITGB4 DST

Sources for Linear Iga Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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