MCID: LCH013
MIFTS: 36

Lichen Planus Pemphigoides

Categories: Rare diseases, Skin diseases

Aliases & Classifications for Lichen Planus Pemphigoides

MalaCards integrated aliases for Lichen Planus Pemphigoides:

Name: Lichen Planus Pemphigoides 53 59 73
Lp Pemphigoides 53 59

Characteristics:

Orphanet epidemiological data:

59
lichen planus pemphigoides
Prevalence: <1/1000000 (Worldwide); Age of onset: All ages;

Classifications:

Orphanet: 59  
Rare skin diseases


External Ids:

Orphanet 59 ORPHA254478
UMLS via Orphanet 74 C0406369
ICD10 via Orphanet 34 L43.8
UMLS 73 C0406369

Summaries for Lichen Planus Pemphigoides

NIH Rare Diseases : 53 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 pemphigoid gestationis. 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, testes and colon, and related phenotypes are hyperkeratosis and hypopigmented streaks

Related Diseases for Lichen Planus Pemphigoides

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

(show all 45)
# Related Disease Score Top Affiliating Genes
1 lichen planus 30.6 COL17A1 DST
2 pemphigoid gestationis 29.8 COL17A1 DST
3 paraneoplastic pemphigus 29.8 COL17A1 DST
4 bullous pemphigoid 29.4 COL17A1 DST ITGB4
5 pemphigus 29.3 COL17A1 DST
6 bullous lichen planus 10.3
7 adenocarcinoma 10.1
8 oral lichen planus 10.1
9 kindler syndrome 10.0 DST ITGB4
10 keratoacanthoma 9.9
11 liposarcoma 9.9
12 palmoplantar keratosis 9.9
13 cellular myxoid liposarcoma 9.9
14 chickenpox 9.9
15 lymphosarcoma 9.9
16 pemphigus vulgaris, familial 9.9
17 lupus erythematosus 9.9
18 hepatitis 9.9
19 hepatitis b 9.9
20 pemphigus vulgaris 9.9
21 cutaneous lupus erythematosus 9.9
22 lymphoma 9.9
23 subacute cutaneous lupus erythematosus 9.9
24 melanoma 9.9
25 colon adenocarcinoma 9.9
26 neurotic excoriation 9.9 COL17A1 DST
27 herpes gestationis 9.9 COL17A1 DST
28 pyloric atresia 9.9 COL17A1 ITGB4
29 epidermolysis bullosa simplex with muscular dystrophy 9.9 COL17A1 DST
30 autoimmune disease of skin and connective tissue 9.9 COL17A1 DST
31 lichen disease 9.8 COL17A1 DST
32 bullous skin disease 9.8 COL17A1 DST
33 ecthyma 9.8 COL17A1 DST
34 subcorneal pustular dermatosis 9.7 COL17A1 DST
35 hypersensitivity reaction disease 9.6 COL17A1 DST
36 linear iga disease 9.4 COL17A1 DST ITGB4
37 vesiculobullous skin disease 9.4 COL17A1 DST ITGB4
38 epidermolysis bullosa acquisita 9.4 COL17A1 DST ITGB4
39 epidermolysis bullosa junctionalis with pyloric atresia 9.4 COL17A1 DST ITGB4
40 cicatricial pemphigoid 9.4 COL17A1 DST ITGB4
41 epidermolysis bullosa, junctional, non-herlitz type 9.4 COL17A1 DST ITGB4
42 junctional epidermolysis bullosa 9.4 COL17A1 DST ITGB4
43 epidermolysis bullosa simplex 9.4 COL17A1 DST ITGB4
44 epidermolysis bullosa 9.3 COL17A1 DST ITGB4
45 skin disease 9.3 COL17A1 DST ITGB4

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:

59 32 (show all 11)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hyperkeratosis 59 32 hallmark (90%) Very frequent (99-80%) HP:0000962
2 hypopigmented streaks 59 32 hallmark (90%) Very frequent (99-80%) HP:0007535
3 lichenification 59 32 hallmark (90%) Very frequent (99-80%) HP:0100725
4 skin vesicle 59 32 hallmark (90%) Very frequent (99-80%) HP:0200037
5 pruritus 59 32 frequent (33%) Frequent (79-30%) HP:0000989
6 blepharitis 59 32 very rare (1%) Very rare (<4-1%) HP:0000498
7 conjunctivitis 59 32 very rare (1%) Very rare (<4-1%) HP:0000509
8 abnormality of the nail 59 32 very rare (1%) Very rare (<4-1%) HP:0001597
9 abnormal blistering of the skin 59 32 very rare (1%) Very rare (<4-1%) HP:0008066
10 abnormality of oral mucosa 59 Frequent (79-30%)
11 abnormal oral mucosa morphology 32 frequent (33%) HP:0011830

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:

41
Skin, Testes, Colon

Publications for Lichen Planus Pemphigoides

Articles related to Lichen Planus Pemphigoides:

(show top 50) (show all 91)
# Title Authors Year
1
Lichen planus pemphigoides. ( 29465781 )
2018
2
Lichen planus pemphigoides. ( 29537153 )
2018
3
Vulvar lichen planus pemphigoides. ( 29234717 )
2017
4
Lichen Planus Pemphigoides Induced by Enalapril: A Case Report and a Review of Literature. ( 29282395 )
2017
5
Bullous lichen planus accompanied by elevation of serum anti-BP180 autoantibody: A possible transitional mechanism to lichen planus pemphigoides. ( 28105695 )
2017
6
A case of lichen planus pemphigoides with palmoplantar keratoderma. ( 29160215 )
2017
7
Pembrolizumab-induzierter Lichen planus pemphigoides bei einem Patienten mit metastasiertem Melanom. ( 28677196 )
2017
8
Pembrolizumab-induced lichen planus pemphigoides in a patient with metastatic melanoma. ( 28622432 )
2017
9
Lichen planus pemphigoides treated with ustekinumab. ( 29360890 )
2017
10
Non-Bullous Lichen Planus Pemphigoides Possibly Induced by Venlafaxine. ( 27124647 )
2016
11
Successful Treatment of Resistant Lichen Planus Pemphigoides with Cyclosporine: A New Hope. ( 26955117 )
2016
12
Lichen planus pemphigoides associated with pregnancy mimicking pemphigoid gestationis. ( 27416093 )
2016
13
Lichen Planus Pemphigoides Presenting Preferentially Over Preexisting Scars: A Rare Instance of Isotopic Phenomenon. ( 26677275 )
2015
14
Oral lichen planus pemphigoides: a series of four cases. ( 25953638 )
2015
15
Evaluation of Mononuclear Cells in Lichen Planus Pemphigoides. ( 26122984 )
2015
16
Lichen planus pemphigoides concomitant with rectal adenocarcinoma: fortuitous or a true association? ( 26394647 )
2015
17
Childhood lichen planus pemphigoides triggered by chickenpox. ( 25593817 )
2014
18
Lichen planus pemphigoides associated with chronic hepatitis B virus infection. ( 25546603 )
2014
19
A case of lichen planus pemphigoides with autoantibodies to the NC16a and C-terminal domains of BP180 and to desmoglein-1. ( 24813536 )
2014
20
Successful treatment of lichen planus pemphigoides using acitretin as monotherapy. ( 25087694 )
2014
21
A case of lichen planus pemphigoides successfully treated with a combination of cyclosporine a and prednisolone. ( 23626545 )
2013
22
Childhood Lichen Planus Pemphigoides: Report of Two Cases Treated Successfully with Systemic Glucocorticoids and Dapsone. ( 24138413 )
2013
23
Lichen planus pemphigoides possibly induced by hormone therapy. ( 24161496 )
2013
24
Lichen planus pemphigoides: four new cases and a review of the literature. ( 23331194 )
2013
25
Lichen planus pemphigoides in a 2-year-old girl: response to treatment with methotrexate. ( 22980277 )
2012
26
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
27
Lichen planus pemphigoides in a child. ( 22645288 )
2012
28
Blisters and plaques on the extremities. What is your diagnosis? Lichen planus pemphigoides. ( 21244376 )
2011
29
Lichen planus pemphigoides induced by a weight reduction drug. ( 21428725 )
2011
30
Heterogeneous disease: a child case of lichen planus pemphigoides triggered by varicella. ( 21453319 )
2011
31
Lichen planus pemphigoides, a possible example of epitope spreading. ( 20382044 )
2010
32
Lichen planus pemphigoides: detection of anti-BP 180 antibodies by ELISA and immunoblotting tests. ( 20337815 )
2010
33
Lichen planus pemphigoides in a 6-year-old child. ( 19086935 )
2009
34
Lichenoid eruption with single plantar blisters: a very rare case of lichen planus pemphigoides. ( 18771440 )
2009
35
Childhood lichen planus pemphigoides: a case report and review of the literature. ( 19840313 )
2009
36
Lichen planus pemphigoides associated with Chinese herbs. ( 18699839 )
2009
37
Captopril-induced lichen planus pemphigoides. ( 18489011 )
2008
38
Lichen planus pemphigoides triggered by narrowband UVB, paracetamol, and ibuprofen, with autoantibodies to 130kDa antigen. ( 18174800 )
2008
39
Clinical and immunopathologic findings in oral lichen planus pemphigoides. ( 17531940 )
2007
40
Lichen planus pemphigoides: a case report and review of the literature. ( 17083865 )
2006
41
Lichen planus pemphigoides associated with ramipril. ( 17184261 )
2006
42
Childhood lichen planus pemphigoides - a rare entity. ( 16405631 )
2006
43
Lichen planus pemphigoides and multiple keratoacanthomas associated with colon adenocarcinoma. ( 15270914 )
2004
44
[Simvastatin-induced lichen planus pemphigoides]. ( 12671581 )
2003
45
Lichen planus pemphigoides evolving into pemphigoid nodularis. ( 14616828 )
2003
46
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
47
Lichen planus pemphigoides: report of two cases. ( 11903670 )
2001
48
Lichen planus pemphigoides with IgG autoantibodies to the 180 kd bullous pemphigoid antigen (type XVII collagen). ( 10607335 )
2000
49
Lichen planus pemphigoides with circulating autoantibodies against 200 and 180 kDa epidermal antigens. ( 10725820 )
2000
50
PUVA-induced lichen planus pemphigoides. ( 10735960 )
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 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 Lichen Planus Pemphigoides

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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