MCID: LCH013
MIFTS: 33

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 53 59 72
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:

ICD10 via Orphanet 34 L43.8
UMLS via Orphanet 73 C0406369
Orphanet 59 ORPHA254478
UMLS 72 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 bullous pemphigoid. 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 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 top 50) (show all 70)
# Related Disease Score Top Affiliating Genes
1 lichen planus 31.7 DST COL17A1
2 bullous pemphigoid 30.6 ITGB4 DST COL17A1
3 pemphigoid gestationis 30.5 DST COL17A1
4 pemphigus 30.2 DST COL17A1
5 paraneoplastic pemphigus 30.1 DST COL17A1
6 epidermolysis bullosa 29.8 ITGB4 DST COL17A1
7 epidermolysis bullosa acquisita 29.8 ITGB4 DST COL17A1
8 linear iga disease 29.7 ITGB4 DST COL17A1
9 skin disease 29.7 ITGB4 DST COL17A1
10 cicatricial pemphigoid 29.6 ITGB4 DST COL17A1
11 bullous lichen planus 10.6
12 ocular cicatricial pemphigoid 10.4
13 oral lichen planus 10.4
14 pemphigus gestationis 10.4
15 pemphigus vulgaris, familial 10.3
16 erythema multiforme 10.3
17 adenocarcinoma 10.3
18 chickenpox 10.3
19 autoimmune disease 10.1
20 hypercholesterolemia, familial, 1 10.1
21 leukemia, chronic lymphocytic 10.1
22 lichen sclerosus et atrophicus 10.1
23 porphyria cutanea tarda 10.1
24 small cell cancer of the lung 10.1
25 lung cancer 10.1
26 myasthenia gravis 10.1
27 severe cutaneous adverse reaction 10.1
28 cutaneous lupus erythematosus 10.1
29 exanthem 10.1
30 colitis 10.1
31 dermatomyositis 10.1
32 lymphocytic leukemia 10.1
33 porphyria 10.1
34 urticaria 10.1
35 cheilitis 10.1
36 rectum adenocarcinoma 10.1
37 hepatitis b 10.1
38 hepatitis 10.1
39 colon adenocarcinoma 10.1
40 liposarcoma 10.1
41 palmoplantar keratosis 10.1
42 pustulosis of palm and sole 10.1
43 cellular myxoid liposarcoma 10.1
44 ulcerative colitis 10.1
45 psoriasis 10.1
46 parapsoriasis 10.1
47 guttate psoriasis 10.1
48 leukemia, b-cell, chronic 10.1
49 lichen sclerosus 10.1
50 prurigo nodularis 10.1

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

41
Skin, Testes, Colon, Lung

Publications for Lichen Planus Pemphigoides

Articles related to Lichen Planus Pemphigoides:

(show top 50) (show all 131)
# Title Authors PMID Year
1
Lichen planus pemphigoides with complete clinical response after ciclosporin therapy. 38
30848484 2019
2
Oral lichen planus: A disease or a spectrum of tissue reactions? Types, causes, diagnostic algorhythms, prognosis, management strategies. 38
31090143 2019
3
Review of autoimmune blistering diseases: the Pemphigoid diseases. 38
31087464 2019
4
Lichen planus pemphigoides developing from patient with non-small-cell lung cancer treated with nivolumab. 38
31062404 2019
5
Lichen Planus Pemphigoides Mimicking Toxic Epidermal Necrolysis. 38
31145106 2019
6
Nivolumab-induced lichen planus pemphigoides. 38
31116807 2019
7
Lichen Planus Pemphigoides: From Lichenoid Inflammation to Autoantibody-Mediated Blistering. 38
31312198 2019
8
Lichen planus pemphigoides. 38
29537153 2018
9
Lichen planus pemphigoides. 38
29465781 2018
10
A case of lichen planus pemphigoides with palmoplantar keratoderma. 38
29160215 2018
11
Vulvar lichen planus pemphigoides. 38
29234717 2017
12
Lichen planus pemphigoides treated with ustekinumab. 38
29360890 2017
13
Lichen Planus Pemphigoides Induced by Enalapril: A Case Report and a Review of Literature. 38
29282395 2017
14
Pembrolizumab-induced lichen planus pemphigoides in a patient with metastatic melanoma. 38
28622432 2017
15
Pembrolizumab-induzierter Lichen planus pemphigoides bei einem Patienten mit metastasiertem Melanom. 38
28677196 2017
16
Bullous lichen planus accompanied by elevation of serum anti-BP180 autoantibody: A possible transitional mechanism to lichen planus pemphigoides. 38
28105695 2017
17
Bullous lichen planus - a review. 38
28539981 2017
18
Lichen planus pemphigoides associated with pregnancy mimicking pemphigoid gestationis. 38
27416093 2016
19
Clinical, demographic and immunopathological spectrum of subepidermal autoimmune bullous diseases at a tertiary center: A 1-year audit. 38
27088957 2016
20
Non-Bullous Lichen Planus Pemphigoides Possibly Induced by Venlafaxine. 38
27124647 2016
21
Evaluation of Mononuclear Cells in Lichen Planus Pemphigoides. 38
26122984 2016
22
Successful Treatment of Resistant Lichen Planus Pemphigoides with Cyclosporine: A New Hope. 38
26955117 2016
23
Lichen planus pemphigoides associated with chronic hepatitis B virus infection. 38
25546603 2015
24
Lichen Planus Pemphigoides Presenting Preferentially Over Preexisting Scars: A Rare Instance of Isotopic Phenomenon. 38
26677275 2015
25
Lichen planus pemphigoides concomitant with rectal adenocarcinoma: fortuitous or a true association? 38
26394647 2015
26
Update on lichen planus and its clinical variants. 38
28491978 2015
27
Oral lichen planus pemphigoides: a series of four cases. 38
25953638 2015
28
Childhood lichen planus pemphigoides triggered by chickenpox. 38
25593817 2014
29
Immunofluorescence testing in the diagnosis of autoimmune blistering diseases: overview of 10-year experience. 38
25387492 2014
30
Childhood lichen planus pemphigoides: report of two cases treated successfully with systemic glucocorticoids and dapsone. 38
24138413 2014
31
A case of lichen planus pemphigoides with autoantibodies to the NC16a and C-terminal domains of BP180 and to desmoglein-1. 38
24813536 2014
32
Successful treatment of lichen planus pemphigoides using acitretin as monotherapy. 38
25087694 2014
33
Lichen planus pemphigoides possibly induced by hormone therapy. 38
24161496 2013
34
Lichen planus pemphigoides: four new cases and a review of the literature. 38
23331194 2013
35
A case of lichen planus pemphigoides successfully treated with a combination of cyclosporine a and prednisolone. 38
23626545 2013
36
Atypical subacute cutaneous lupus erythematosus presenting as lichen planus pemphigoides with autoantibodies to C-terminus of BP180, desmoglein 1 and SS-A/Ro antigen. 38
22413755 2012
37
Lichen planus pemphigoides in a 2-year-old girl: response to treatment with methotrexate. 38
22980277 2012
38
Lichen planus pemphigoides in a child. 38
22645288 2012
39
Pemphigoid diseases: pathogenesis, diagnosis, and treatment. 38
21923615 2012
40
Lichen planus pemphigoides induced by a weight reduction drug. 38
21428725 2011
41
Narrowband UVB-induced lichen planus pemphigoide. 38
25386295 2011
42
Heterogeneous disease: a child case of lichen planus pemphigoides triggered by varicella. 38
21453319 2011
43
Blisters and plaques on the extremities. What is your diagnosis? Lichen planus pemphigoides. 38
21244376 2011
44
Lichen planus pemphigoides: detection of anti-BP 180 antibodies by ELISA and immunoblotting tests. 38
20337815 2010
45
Lichen planus pemphigoides, a possible example of epitope spreading. 38
20382044 2010
46
A case of psoriasis worsened by atorvastatin. 38
21886735 2009
47
Childhood lichen planus pemphigoides: a case report and review of the literature. 38
19840313 2009
48
Lichenoid eruption with single plantar blisters: a very rare case of lichen planus pemphigoides. 38
18771440 2009
49
The utility of the DNA microarray scanner to simplify the immunofluorescence evaluation of autoimmune bullous diseases. 38
19384060 2009
50
Lichen planus pemphigoides associated with Chinese herbs. 38
18699839 2009

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 ITGB4 DST COL17A1
2 basement membrane GO:0005604 9.16 DST COL17A1
3 cell leading edge GO:0031252 8.96 ITGB4 DST
4 hemidesmosome GO:0030056 8.8 ITGB4 DST COL17A1

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 ITGB4 COL17A1
2 integrin-mediated signaling pathway GO:0007229 9.32 ITGB4 DST
3 cell-matrix adhesion GO:0007160 9.26 ITGB4 COL17A1
4 response to wounding GO:0009611 9.16 ITGB4 DST
5 cell motility GO:0048870 8.96 ITGB4 DST
6 hemidesmosome assembly GO:0031581 8.8 ITGB4 DST COL17A1

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 ITGB4 DST

Sources for Lichen Planus Pemphigoides

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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