MCID: MLG141
MIFTS: 36

Malignant Atrophic Papulosis

Categories: Blood diseases, Bone diseases, Cancer diseases, Cardiovascular diseases, Rare diseases, Skin diseases
Data Licensing
For inquiries, contact:

Aliases & Classifications for Malignant Atrophic Papulosis

MalaCards integrated aliases for Malignant Atrophic Papulosis:

Name: Malignant Atrophic Papulosis 57 19 58 71 75
Degos Disease 57 19 58 75 53
Papulosis Atrophican Maligna 19 58
Kohlmeier-Degos Disease 19 58
Köhlmeier-Degos-Delort-Tricort Syndrome 19
Kohlmeier-Degos-Delort-Tricort Syndrome 58
Degos's Malignant Atrophic Papulosis 19
Papulosis, Malignant Atrophic 57
Atrophic Papulosis, Malignant 19
Köhlmeier-Degos Disease 19

Characteristics:


Inheritance:

Autosomal dominant 58 57

Prevelance:

<1/1000000 (Worldwide) 58

Age Of Onset:

Adult 58

Age Of Death:

adult 58

OMIM®:

57 (Updated 24-Oct-2022)
Miscellaneous:
male-to-female ratio 3 to 1
usually occurs in young adults


Classifications:

Orphanet: 58  
Rare systemic and rhumatological diseases
Rare skin diseases


Summaries for Malignant Atrophic Papulosis

GARD: 19 Degos disease is a rare blood vessel disorder. This slows or stops the flow of blood through the affected vessels. Severity of symptoms depends on the extent and location of the affected blood vessels. Some individuals with Degos disease have isolated skin involvement and develop porcelain-white macules on their skin. Other individuals have more wide spread disease. The cause of this condition is currently unknown.

MalaCards based summary: Malignant Atrophic Papulosis, also known as degos disease, is related to dowling-degos disease and dowling-degos disease 1. An important gene associated with Malignant Atrophic Papulosis is APOH (Apolipoprotein H). Affiliated tissues include skin, bone and eye, and related phenotypes are telangiectasia of the skin and papule

Orphanet: 58 Malignant atrophic papulosis (MAP) is a rare, chronic, thrombo-obliterative vasculopathy characterized by papular skin lesions with central porcelain-white atrophy and a surrounding teleangiectatic rim. Systemic lesions may affect the gastrointestinal tract and the central nervous system (CNS) and are potentially lethal.

Wikipedia: 75 Degos disease, also known as Köhlmeier-Degos disease or malignant atrophic papulosis, is an extremely... more...

More information from OMIM: 602248

Related Diseases for Malignant Atrophic Papulosis

Diseases related to Malignant Atrophic Papulosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 159)
# Related Disease Score Top Affiliating Genes
1 dowling-degos disease 11.9
2 dowling-degos disease 1 11.6
3 dowling-degos disease 2 11.6
4 dowling-degos disease 4 11.6
5 acne inversa, familial, 2, with or without dowling-degos disease 11.6
6 erythrokeratoderma ''en cocardes'' 11.5
7 dowling-degos disease 3 11.2
8 hidradenitis suppurativa 10.6
9 hidradenitis 10.6
10 skin disease 10.6
11 contractures, pterygia, and spondylocarpotarsal fusion syndrome 1a 10.6
12 peritonitis 10.5
13 reticulate acropigmentation of kitamura 10.5
14 systemic lupus erythematosus 10.4
15 lupus erythematosus 10.4
16 systemic scleroderma 10.4
17 vasculitis 10.4
18 acne 10.3
19 scleroderma, familial progressive 10.3
20 systemic lupus erythematosus 1 10.3
21 ptosis 10.3
22 intestinal perforation 10.3
23 connective tissue disease 10.3
24 rare genetic skin disease 10.2
25 acanthosis nigricans 10.2
26 constrictive pericarditis 10.2
27 keratosis 10.2
28 kearns-sayre syndrome 10.2
29 vascular disease 10.2
30 optic nerve disease 10.2
31 purpura 10.2
32 polyradiculopathy 10.2
33 thrombotic microangiopathy 10.2
34 melanosis, neurocutaneous 10.2
35 autoimmune disease 10.1
36 epidermoid cysts 10.1
37 psoriasis 1 10.1
38 keratosis, seborrheic 10.1
39 psoriasis 2 10.1
40 psoriasis 7 10.1
41 psoriasis 11 10.1
42 psoriasis 13 10.1
43 dyschromatosis universalis hereditaria 10.1
44 psoriasis 10.1
45 sarcoidosis 1 10.1
46 thrombocytopenic purpura, autoimmune 10.1
47 3-methylglutaconic aciduria, type iii 10.1
48 stroke, ischemic 10.1
49 muscular dystrophy, congenital merosin-deficient, 1a 10.1
50 sarcoidosis 2 10.1

Graphical network of the top 20 diseases related to Malignant Atrophic Papulosis:



Diseases related to Malignant Atrophic Papulosis

Symptoms & Phenotypes for Malignant Atrophic Papulosis

Human phenotypes related to Malignant Atrophic Papulosis:

58 30 (show all 38)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 telangiectasia of the skin 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0100585
2 papule 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0200034
3 dermal atrophy 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0004334
4 nausea and vomiting 58 30 Frequent (33%) Frequent (79-30%)
HP:0002017
5 fatigue 58 30 Frequent (33%) Frequent (79-30%)
HP:0012378
6 abdominal pain 58 30 Frequent (33%) Frequent (79-30%)
HP:0002027
7 gastrointestinal hemorrhage 58 30 Frequent (33%) Frequent (79-30%)
HP:0002239
8 weight loss 58 30 Frequent (33%) Frequent (79-30%)
HP:0001824
9 gastrointestinal infarctions 58 30 Frequent (33%) Frequent (79-30%)
HP:0005244
10 muscle flaccidity 58 30 Frequent (33%) Frequent (79-30%)
HP:0010547
11 intestinal perforation 58 30 Frequent (33%) Frequent (79-30%)
HP:0031368
12 ptosis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000508
13 diplopia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000651
14 cataract 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000518
15 cranial nerve paralysis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0006824
16 myocardial infarction 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001658
17 abnormal myocardium morphology 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001637
18 vertigo 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002321
19 peripheral neuropathy 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0009830
20 migraine 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002076
21 chest pain 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0100749
22 arterial thrombosis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0004420
23 respiratory failure 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002878
24 pleural effusion 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002202
25 abnormal pericardium morphology 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001697
26 peritonitis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002586
27 amaurosis fugax 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0100576
28 intestinal fistula 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0100819
29 pain insensitivity 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0007021
30 abnormality of the optic nerve 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000587
31 ischemic stroke 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002140
32 arteritis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0012089
33 abnormality of the lower urinary tract 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0010936
34 seizure 30 Occasional (7.5%) HP:0001250
35 seizures 58 Occasional (29-5%)
36 stroke 30 HP:0001297
37 abnormal conjunctiva morphology 30 HP:0000502
38 constrictive pericarditis 30 HP:0002563

Symptoms via clinical synopsis from OMIM®:

57 (Updated 24-Oct-2022)
Abdomen Gastrointestinal:
gastrointestinal infarctions
gastrointestinal bleeding

Head And Neck Eyes:
avascular patches on conjunctiva and other eye regions

Skin Nails Hair Skin Histology:
wedge-shaped necrosis from epidermis through dermis

Respiratory Lung:
pleural effusion

Skin Nails Hair Skin:
multiple asymptomatic papules with atrophic white scar-like centers surrounded by telangiectatic borders

Neurologic Central Nervous System:
cns infarctions

Clinical features from OMIM®:

602248 (Updated 24-Oct-2022)

Drugs & Therapeutics for Malignant Atrophic Papulosis

Search Clinical Trials, NIH Clinical Center for Malignant Atrophic Papulosis

Genetic Tests for Malignant Atrophic Papulosis

Anatomical Context for Malignant Atrophic Papulosis

Organs/tissues related to Malignant Atrophic Papulosis:

MalaCards : Skin, Bone, Eye, Small Intestine, Spinal Cord, Endothelial, Liver

Publications for Malignant Atrophic Papulosis

Articles related to Malignant Atrophic Papulosis:

(show top 50) (show all 514)
# Title Authors PMID Year
1
Malignant atrophic papulosis (Degos' disease) involving three generations of a family. 62 57
9308565 1997
2
[2 families with malignant atrophic papulosis (Degos disease)]. 62 57
3279324 1988
3
Six cases of malignant atrophic papulosis (Degos' disease) occurring in one family. 62 57
6487548 1984
4
Familial malignant atrophic papulosis. 62 57
6733962 1984
5
Malignant atrophic papulosis (Degos' disease). Two cases occurring in the same family. 62 57
5359899 1969
6
Degos' disease (malignant papulosis). Report of three cases with clues to etiology. 62 57
4176652 1968
7
Malignant atrophic papulosis: endocardial involvement and positive anticardiolipin antibodies. 53 62
16684293 2006
8
Inflammation and thrombo-occlusive vessel signalling in benign atrophic papulosis (Köhlmeier-Degos disease). 62
35748122 2022
9
Atrophic papulosis (Köhlmeier-Degos disease) revisited: a cross-sectional study on 105 patients. 62
35610757 2022
10
Interferon receptor dysfunction in a child with malignant atrophic papulosis and CNS involvement. 62
35841902 2022
11
Exploring the pathophysiologic basis of constrictive pericarditis of Kohlmeier Degos disease: A case series and review of the literature. 62
35588602 2022
12
A case report of Dowling-Degos disease caused by POFUT1 exon deletion with possible coexistent CARD14 mutation-related psoriasis. 62
35340079 2022
13
Acquired Dermal Macular Hyperpigmentation Mimicking Dowling Degos Disease: A Case Report. 62
36148207 2022
14
A Particular Bicentric Structure in Dermoscopic Demonstration of Degos Disease. 62
36159151 2022
15
Laparoscopic detection of malignant atrophic papulosis (MAP) in a Paediatric patient. 62
35857385 2022
16
Diffused Degos disease treated with rivaroxaban. 62
35707869 2022
17
Follicular Dowling-Degos Disease Camouflaged as Comedones: A Case Report and Literature Review. 62
35865417 2022
18
Degos disease complicated by constrictive pericarditis in remote phase: a case report. 62
35365165 2022
19
Gastrointestinal Kohlmeier-Degos disease: a narrative review. 62
35443671 2022
20
Dowling-Degos Disease Presenting With Associated Epidermal Inclusion Cysts: A Case Report and Review of the Literature. 62
34816802 2022
21
Evolutionary distinct roles of γ-secretase subunit nicastrin in zebrafish and humans. 62
35016821 2022
22
Dissecting the Roles of Innate Cells in the Skin and Intractable Skin Diseases. 62
35753764 2022
23
Neurological Involvement in Malignant Atrophic Papulosis: A Comprehensive Review of Literature. 62
35263846 2022
24
A fatal case of malignant atrophic papulosis in a pediatric patient. 62
34935194 2022
25
Novel deletion of the POFUT1 gene associated with multiple seborrheic keratosis Dowling-Degos disease. 62
34524711 2021
26
Degos disease with renal cell carcinoma. 62
34184287 2021
27
Classic Dowling Degos disease: a rare genodermatosis. 62
31195785 2021
28
A Case of Malignant Atrophic Papulosis With Multiple Complications. 62
34074813 2021
29
Clinico-Pathological Features in Köhlmeier-Degos Disease with Cutaneous and Neurological Involvement. 62
35359530 2021
30
Malignant atrophic papulosis (Degos disease). 62
34570365 2021
31
Follicular Dowling-Degos Disease with Hidradenitis Suppurativa: A Case Report and Review of the Literature. 62
35082614 2021
32
Dowling-Degos disease: a review. 62
33368260 2021
33
Co-occurrence of Dowling-Degos disease and pemphigus vulgaris. 62
33660255 2021
34
Clinical manifestations and treatment outcomes in degos disease: a systematic review. 62
33914972 2021
35
Middle of the breasts pigmentation in a pedigree with POFUT1-related Dowling-Degos disease, expansion of the phenotype. 62
34377138 2021
36
Exacerbation of Galli-Galli Disease Following Dialysis Treatment: A Case Report and Review of Aggravating Factors. 62
34249550 2021
37
Diseases related to Notch glycosylation. 62
33341260 2021
38
Fucosylated Proteome Profiling Identifies a Fucosylated, Non-Ribosomal, Stress-Responsive Species of Ribosomal Protein S3. 62
34070332 2021
39
Clinical and laboratory prognosticators of atrophic papulosis (Degos disease): a systematic review. 62
33957947 2021
40
Follicular Dowling Degos Disease with Lichen Planus in Two Siblings: A Rare Association. 62
34211919 2021
41
Degos-like lesions as a cutaneous manifestation of cytomegalovirus infection: A rare and serious complication in a patient with drug-induced hypersensitivity syndrome. 62
33305840 2021
42
Co-occurrence of Ichthyosis Vulgaris, Dowling-Degos Disease, and Hidradenitis Suppuritiva in Same Patient: An Association or Coincidence? 62
34188288 2021
43
Multiomics Integration in Skin Diseases with Alterations in Notch Signaling Pathway: PlatOMICs Phase 1 Deployment. 62
33546374 2021
44
Comorbid acne inversa and Dowling-Degos disease due to a single NCSTN mutation: is there enough evidence? Reply from the authors. 62
32895925 2021
45
Comorbid acne inversa and Dowling-Degos disease due to a single NCSTN mutation: is there enough evidence? 62
32894577 2021
46
PSENEN Mutation in Coexistent Hidradenitis Suppurativa and Dowling-Degos Disease. 62
33768038 2021
47
Follicular Dowling-Degos Disease and Blepharophimosis-Ptosis-Epicanthus Inversus Syndrome: A Chance or Significant Association. 62
33911305 2021
48
Degos Disease (Malignant Atrophic Papulosis) With Granular IgM on Direct Immunofluorescence. 62
33604213 2021
49
Case Report: Pediatric Malignant Atrophic Papulosis With Small Bowel Perforation and Positivity of Anticardiolipin Antibody. 62
34956979 2021
50
Structure, function, and pathology of protein O-glucosyltransferases. 62
33436558 2021

Variations for Malignant Atrophic Papulosis

Expression for Malignant Atrophic Papulosis

Search GEO for disease gene expression data for Malignant Atrophic Papulosis.

Pathways for Malignant Atrophic Papulosis

GO Terms for Malignant Atrophic Papulosis

Sources for Malignant Atrophic Papulosis

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 24-Oct-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
Content
Loading form....