MCID: LCH013
MIFTS: 36

Lichen Planus Pemphigoides

Categories: Immune diseases, Rare diseases, Skin diseases

Aliases & Classifications for Lichen Planus Pemphigoides

MalaCards integrated aliases for Lichen Planus Pemphigoides:

Name: Lichen Planus Pemphigoides 54 60 74
Lp Pemphigoides 54 60

Characteristics:

Orphanet epidemiological data:

60
lichen planus pemphigoides
Prevalence: <1/1000000 (Worldwide); Age of onset: All ages;

Classifications:

Orphanet: 60  
Rare skin diseases


External Ids:

ICD10 via Orphanet 35 L43.8
UMLS via Orphanet 75 C0406369
Orphanet 60 ORPHA254478
UMLS 74 C0406369

Summaries for Lichen Planus Pemphigoides

NIH Rare Diseases : 54 Lichen planus pemphigoides (LPP) is a rare cross-over syndrome between lichen planus and bullous pemphigoid. Like other forms of lichen planus, it is characterized by a skin rash (shiny, flat-topped, firm bumps that are a purple color and vary from pin point size to larger than a centimeter); however, people affected by LPP subsequently develop blisters on both the lichen planus skin lesions and on normal skin. The exact underlying cause of LPP is currently unknown, but some studies suggest that it may be associated with an allergic or immune reaction. Treatment for LPP is based on the signs and symptoms present in each person.

MalaCards based summary : Lichen Planus Pemphigoides, also known as lp pemphigoides, is related to lichen planus and pemphigus. An important gene associated with Lichen Planus Pemphigoides is DST (Dystonin), and among its related pathways/superpathways are Collagen chain trimerization and Degradation of the extracellular matrix. Affiliated tissues include skin, colon and testes, and related phenotypes are hyperkeratosis and skin vesicle

Related Diseases for Lichen Planus Pemphigoides

Diseases related to Lichen Planus Pemphigoides via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 46)
# Related Disease Score Top Affiliating Genes
1 lichen planus 31.6 COL17A1 DST
2 pemphigus 30.2 COL17A1 DST
3 paraneoplastic pemphigus 30.2 COL17A1 DST
4 bullous pemphigoid 30.1 COL17A1 DST ITGB4
5 pemphigoid gestationis 30.0 COL17A1 DST
6 bullous lichen planus 10.4
7 adenocarcinoma 10.2
8 oral lichen planus 10.2
9 pemphigus vulgaris, familial 10.1
10 hepatitis 10.1
11 hepatitis b 10.1
12 pemphigus vulgaris 10.1
13 pemphigus gestationis 10.1
14 cutaneous lupus erythematosus 10.1
15 lymphoma 10.1
16 melanoma 10.1
17 rectum adenocarcinoma 10.1
18 colon adenocarcinoma 10.1
19 liposarcoma 10.1
20 palmoplantar keratosis 10.1
21 cellular myxoid liposarcoma 10.1
22 chickenpox 10.1
23 lymphosarcoma 10.1
24 lupus erythematosus 10.1
25 subacute cutaneous lupus erythematosus 10.1
26 bullous skin disease 10.0 COL17A1 DST
27 pyloric atresia 10.0 COL17A1 ITGB4
28 kindler syndrome 10.0 DST ITGB4
29 neurotic excoriation 9.9 COL17A1 DST
30 epidermolysis bullosa simplex with muscular dystrophy 9.9 COL17A1 DST
31 autoimmune disease of skin and connective tissue 9.9 COL17A1 DST
32 lichen disease 9.9 COL17A1 DST
33 ecthyma 9.9 COL17A1 DST
34 cellulitis 9.9 COL17A1 DST
35 subcorneal pustular dermatosis 9.8 COL17A1 DST
36 linear iga disease 9.7 COL17A1 DST ITGB4
37 vesiculobullous skin disease 9.7 COL17A1 DST ITGB4
38 skin disease 9.7 COL17A1 DST ITGB4
39 epidermolysis bullosa acquisita 9.7 COL17A1 DST ITGB4
40 epidermolysis bullosa 9.7 COL17A1 DST ITGB4
41 cicatricial pemphigoid 9.7 COL17A1 DST ITGB4
42 epidermolysis bullosa junctionalis with pyloric atresia 9.7 COL17A1 DST ITGB4
43 epidermolysis bullosa, junctional, non-herlitz type 9.7 COL17A1 DST ITGB4
44 junctional epidermolysis bullosa 9.7 COL17A1 DST ITGB4
45 epidermolysis bullosa simplex 9.7 COL17A1 DST ITGB4
46 autoimmune disease of musculoskeletal system 9.6 COL17A1 DST

Graphical network of the top 20 diseases related to Lichen Planus Pemphigoides:



Diseases related to Lichen Planus Pemphigoides

Symptoms & Phenotypes for Lichen Planus Pemphigoides

Human phenotypes related to Lichen Planus Pemphigoides:

60 33 (show all 11)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hyperkeratosis 60 33 hallmark (90%) Very frequent (99-80%) HP:0000962
2 skin vesicle 60 33 hallmark (90%) Very frequent (99-80%) HP:0200037
3 lichenification 60 33 hallmark (90%) Very frequent (99-80%) HP:0100725
4 hypopigmented streaks 60 33 hallmark (90%) Very frequent (99-80%) HP:0007535
5 pruritus 60 33 frequent (33%) Frequent (79-30%) HP:0000989
6 abnormal oral mucosa morphology 33 frequent (33%) HP:0011830
7 abnormality of the nail 60 33 very rare (1%) Very rare (<4-1%) HP:0001597
8 abnormal blistering of the skin 60 33 very rare (1%) Very rare (<4-1%) HP:0008066
9 blepharitis 60 33 very rare (1%) Very rare (<4-1%) HP:0000498
10 conjunctivitis 60 33 very rare (1%) Very rare (<4-1%) HP:0000509
11 abnormality of oral mucosa 60 Frequent (79-30%)

Drugs & Therapeutics for Lichen Planus Pemphigoides

Search Clinical Trials , NIH Clinical Center for Lichen Planus Pemphigoides

Genetic Tests for Lichen Planus Pemphigoides

Anatomical Context for Lichen Planus Pemphigoides

MalaCards organs/tissues related to Lichen Planus Pemphigoides:

42
Skin, Colon, Testes

Publications for Lichen Planus Pemphigoides

Articles related to Lichen Planus Pemphigoides:

(show top 50) (show all 92)
# Title Authors Year
1
Lichen planus pemphigoides with complete clinical response after ciclosporin therapy. ( 30848484 )
2019
2
Lichen planus pemphigoides. ( 29465781 )
2018
3
Lichen planus pemphigoides. ( 29537153 )
2018
4
Vulvar lichen planus pemphigoides. ( 29234717 )
2017
5
Lichen Planus Pemphigoides Induced by Enalapril: A Case Report and a Review of Literature. ( 29282395 )
2017
6
Bullous lichen planus accompanied by elevation of serum anti-BP180 autoantibody: A possible transitional mechanism to lichen planus pemphigoides. ( 28105695 )
2017
7
A case of lichen planus pemphigoides with palmoplantar keratoderma. ( 29160215 )
2017
8
Pembrolizumab-induzierter Lichen planus pemphigoides bei einem Patienten mit metastasiertem Melanom. ( 28677196 )
2017
9
Pembrolizumab-induced lichen planus pemphigoides in a patient with metastatic melanoma. ( 28622432 )
2017
10
Lichen planus pemphigoides treated with ustekinumab. ( 29360890 )
2017
11
Non-Bullous Lichen Planus Pemphigoides Possibly Induced by Venlafaxine. ( 27124647 )
2016
12
Successful Treatment of Resistant Lichen Planus Pemphigoides with Cyclosporine: A New Hope. ( 26955117 )
2016
13
Lichen planus pemphigoides associated with pregnancy mimicking pemphigoid gestationis. ( 27416093 )
2016
14
Lichen Planus Pemphigoides Presenting Preferentially Over Preexisting Scars: A Rare Instance of Isotopic Phenomenon. ( 26677275 )
2015
15
Oral lichen planus pemphigoides: a series of four cases. ( 25953638 )
2015
16
Evaluation of Mononuclear Cells in Lichen Planus Pemphigoides. ( 26122984 )
2015
17
Lichen planus pemphigoides concomitant with rectal adenocarcinoma: fortuitous or a true association? ( 26394647 )
2015
18
Childhood lichen planus pemphigoides triggered by chickenpox. ( 25593817 )
2014
19
Lichen planus pemphigoides associated with chronic hepatitis B virus infection. ( 25546603 )
2014
20
A case of lichen planus pemphigoides with autoantibodies to the NC16a and C-terminal domains of BP180 and to desmoglein-1. ( 24813536 )
2014
21
Successful treatment of lichen planus pemphigoides using acitretin as monotherapy. ( 25087694 )
2014
22
A case of lichen planus pemphigoides successfully treated with a combination of cyclosporine a and prednisolone. ( 23626545 )
2013
23
Childhood Lichen Planus Pemphigoides: Report of Two Cases Treated Successfully with Systemic Glucocorticoids and Dapsone. ( 24138413 )
2013
24
Lichen planus pemphigoides possibly induced by hormone therapy. ( 24161496 )
2013
25
Lichen planus pemphigoides: four new cases and a review of the literature. ( 23331194 )
2013
26
Lichen planus pemphigoides in a 2-year-old girl: response to treatment with methotrexate. ( 22980277 )
2012
27
Atypical subacute cutaneous lupus erythematosus presenting as lichen planus pemphigoides with autoantibodies to C-terminus of BP180, desmoglein 1 and SS-A/Ro antigen. ( 22413755 )
2012
28
Lichen planus pemphigoides in a child. ( 22645288 )
2012
29
Blisters and plaques on the extremities. What is your diagnosis? Lichen planus pemphigoides. ( 21244376 )
2011
30
Lichen planus pemphigoides induced by a weight reduction drug. ( 21428725 )
2011
31
Heterogeneous disease: a child case of lichen planus pemphigoides triggered by varicella. ( 21453319 )
2011
32
Lichen planus pemphigoides, a possible example of epitope spreading. ( 20382044 )
2010
33
Lichen planus pemphigoides: detection of anti-BP 180 antibodies by ELISA and immunoblotting tests. ( 20337815 )
2010
34
Lichen planus pemphigoides in a 6-year-old child. ( 19086935 )
2009
35
Lichenoid eruption with single plantar blisters: a very rare case of lichen planus pemphigoides. ( 18771440 )
2009
36
Childhood lichen planus pemphigoides: a case report and review of the literature. ( 19840313 )
2009
37
Lichen planus pemphigoides associated with Chinese herbs. ( 18699839 )
2009
38
Captopril-induced lichen planus pemphigoides. ( 18489011 )
2008
39
Lichen planus pemphigoides triggered by narrowband UVB, paracetamol, and ibuprofen, with autoantibodies to 130kDa antigen. ( 18174800 )
2008
40
Clinical and immunopathologic findings in oral lichen planus pemphigoides. ( 17531940 )
2007
41
Lichen planus pemphigoides: a case report and review of the literature. ( 17083865 )
2006
42
Lichen planus pemphigoides associated with ramipril. ( 17184261 )
2006
43
Childhood lichen planus pemphigoides - a rare entity. ( 16405631 )
2006
44
Lichen planus pemphigoides and multiple keratoacanthomas associated with colon adenocarcinoma. ( 15270914 )
2004
45
[Simvastatin-induced lichen planus pemphigoides]. ( 12671581 )
2003
46
Lichen planus pemphigoides evolving into pemphigoid nodularis. ( 14616828 )
2003
47
Paraneoplastic pemphigus in association with a retroperitoneal Castleman's disease presenting with a lichen planus pemphigoides-like eruption. A case report and review of literature. ( 11251576 )
2001
48
Lichen planus pemphigoides: report of two cases. ( 11903670 )
2001
49
Lichen planus pemphigoides with IgG autoantibodies to the 180 kd bullous pemphigoid antigen (type XVII collagen). ( 10607335 )
2000
50
Lichen planus pemphigoides with circulating autoantibodies against 200 and 180 kDa epidermal antigens. ( 10725820 )
2000

Variations for Lichen Planus Pemphigoides

Expression for Lichen Planus Pemphigoides

Search GEO for disease gene expression data for Lichen Planus Pemphigoides.

Pathways for Lichen Planus Pemphigoides

GO Terms for Lichen Planus Pemphigoides

Cellular components related to Lichen Planus Pemphigoides according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell junction GO:0030054 9.43 COL17A1 DST ITGB4
2 basement membrane GO:0005604 9.16 COL17A1 DST
3 cell leading edge GO:0031252 8.96 DST ITGB4
4 hemidesmosome GO:0030056 8.8 COL17A1 DST ITGB4

Biological processes related to Lichen Planus Pemphigoides 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 Lichen Planus Pemphigoides according to GeneCards Suite gene sharing:

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

Sources for Lichen Planus Pemphigoides

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
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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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