U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Malignant atrophic papulosis

MedGen UID:
113138
•Concept ID:
C0221011
•
Disease or Syndrome
Synonyms: Degos disease; Degos syndrome; Degos's malignant atrophic papulosis; Kohlmeier-Degos disease
SNOMED CT: Malignant atrophic papulosis (400171002); Degos' disease (400171002); Kohlmeier-Degos syndrome (400171002); Lethal cutaneous and gastrointestinal arteriolar thrombosis (400171002); MAP - malignant atrophic papulosis (400171002); Malignant atrophic papulosis of Degos (400171002)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
•Concept ID:
C0443147
•
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Autosomal dominant inheritance (Orphanet)
 
Monarch Initiative: MONDO:0011208
OMIM®: 602248
Orphanet: ORPHA679

Definition

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. [from ORDO]

Clinical features

From HPO
Stroke disorder
MedGen UID:
52522
•Concept ID:
C0038454
•
Disease or Syndrome
Sudden impairment of blood flow to a part of the brain due to occlusion or rupture of an artery to the brain.
See: Feature record | Search on this feature
Gastrointestinal hemorrhage
MedGen UID:
8971
•Concept ID:
C0017181
•
Pathologic Function
Hemorrhage affecting the gastrointestinal tract.
See: Feature record | Search on this feature
Gastrointestinal infarctions
MedGen UID:
463581
•Concept ID:
C3152231
•
Finding
See: Feature record | Search on this feature
Pleural effusion
MedGen UID:
10805
•Concept ID:
C0032227
•
Pathologic Function
The presence of an excessive amount of fluid in the pleural cavity.
See: Feature record | Search on this feature
Constrictive pericarditis
MedGen UID:
18378
•Concept ID:
C0031048
•
Disease or Syndrome
Presence of a thickened, fibrotic pericardium that forms a non-compliant shell around the heart, and resulting from chronic inflammation of the pericardium.
See: Feature record | Search on this feature
Abnormal conjunctiva morphology
MedGen UID:
871353
•Concept ID:
C4025847
•
Anatomical Abnormality
An abnormality of the conjunctiva.
See: Feature record | Search on this feature
Papule
MedGen UID:
507324
•Concept ID:
C0332563
•
Finding
A circumscribed, solid elevation of skin with no visible fluid, varying in size from a pinhead to less than 10mm in diameter at the widest point.
See: Feature record | Search on this feature
Show allHide all

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant atrophic papulosis
Follow this link to review classifications for Malignant atrophic papulosis in Orphanet.

Recent clinical studies

Etiology

Atrophic papulosis (Köhlmeier-Degos disease) in children and adolescents-A cross-sectional study and literature review.
Zouboulis CC, Kaleta KP, Broniatowska E, Jarienė V, Nikolakis G
J Eur Acad Dermatol Venereol 2023 Apr;37(4):810-816. Epub 2023 Jan 14 doi: 10.1111/jdv.18844. PMID: 36606530
Inflammation and thrombo-occlusive vessel signalling in benign atrophic papulosis (Köhlmeier-Degos disease).
Zouboulis CC, Theodoridis A, Makrantonaki E
J Eur Acad Dermatol Venereol 2022 Nov;36(11):2195-2198. Epub 2022 Jul 8 doi: 10.1111/jdv.18362. PMID: 35748122
Congenital Degos Disease: Case Report and Dermoscopic Findings.
Calderón-Castrat X, Castro R, Peceros-Escalante J, Villate Caballero M, Chian C, Ballona R
Pediatr Dermatol 2017 May;34(3):e109-e115. Epub 2017 Mar 20 doi: 10.1111/pde.13096. PMID: 28317162
Malignant and benign forms of atrophic papulosis (Köhlmeier-Degos disease): systemic involvement determines the prognosis.
Theodoridis A, Konstantinidou A, Makrantonaki E, Zouboulis CC
Br J Dermatol 2014 Jan;170(1):110-5. doi: 10.1111/bjd.12642. PMID: 24116834
Mesenteric infarction.
Kairaluoma MI, Karkola P, Heikkinen D, Huttunen R, Mokka RE, Larmi TK
Am J Surg 1977 Feb;133(2):188-93. doi: 10.1016/0002-9610(77)90078-2. PMID: 835791

Diagnosis

Atrophic Papulosis.
Robert M, Hot A
Dermatology 2023;239(2):177-187. Epub 2022 Nov 9 doi: 10.1159/000526789. PMID: 36353773
Malignant atrophic papulosis (Degos disease).
Xu Q, Lin M, Zhang L, Chen S, Gong T, Cheng B, Ji C
Australas J Dermatol 2021 Nov;62(4):e586-e588. Epub 2021 Sep 27 doi: 10.1111/ajd.13722. PMID: 34570365
Malignant Atrophic Papulosis.
Mareschal A, Razanamahery J, Aubin F
JAMA Dermatol 2020 Feb 1;156(2):204. doi: 10.1001/jamadermatol.2019.3726. PMID: 31799991
Malignant atrophic papulosis.
Scheinfeld N
Clin Exp Dermatol 2007 Sep;32(5):483-7. doi: 10.1111/j.1365-2230.2007.02497.x. PMID: 17692056
Mesenteric infarction.
Kairaluoma MI, Karkola P, Heikkinen D, Huttunen R, Mokka RE, Larmi TK
Am J Surg 1977 Feb;133(2):188-93. doi: 10.1016/0002-9610(77)90078-2. PMID: 835791

Therapy

Pediatric Malignant Atrophic Papulosis.
Huang YC, Wang JD, Lee FY, Fu LS
Pediatrics 2018 Apr;141(Suppl 5):S481-S484. doi: 10.1542/peds.2016-4206. PMID: 29610176
Malignant atrophic papulosis with motor aphasia and intestinal perforation: A case report and review of published works.
Hu P, Mao Z, Liu C, Hu X, Kang H, Zhou F
J Dermatol 2018 Jun;45(6):723-726. Epub 2018 Mar 8 doi: 10.1111/1346-8138.14280. PMID: 29516548Free PMC Article
Malignant atrophic papulosis (Köhlmeier-Degos disease) - a review.
Theodoridis A, Makrantonaki E, Zouboulis CC
Orphanet J Rare Dis 2013 Jan 14;8:10. doi: 10.1186/1750-1172-8-10. PMID: 23316694Free PMC Article
Malignant atrophic papulosis.
Degos R
Br J Dermatol 1979 Jan;100(1):21-35. doi: 10.1111/j.1365-2133.1979.tb03566.x. PMID: 371654
Malignant atrophic papulosis of Degos. Report of a patient who failed to respond to fibrinolytic therapy.
Howsden SM, Hodge SJ, Herndon JH, Freeman RG
Arch Dermatol 1976 Nov;112(11):1582-8. doi: 10.1001/archderm.112.11.1582. PMID: 791153

Prognosis

Atrophic Papulosis.
Robert M, Hot A
Dermatology 2023;239(2):177-187. Epub 2022 Nov 9 doi: 10.1159/000526789. PMID: 36353773
Neurological Involvement in Malignant Atrophic Papulosis: A Comprehensive Review of Literature.
Yousefi S, Borhani-Haghighi A, Safari A, Shapiro L
Neurol India 2022 Jan-Feb;70(1):5-10. doi: 10.4103/0028-3886.338719. PMID: 35263846
Pediatric Malignant Atrophic Papulosis.
Huang YC, Wang JD, Lee FY, Fu LS
Pediatrics 2018 Apr;141(Suppl 5):S481-S484. doi: 10.1542/peds.2016-4206. PMID: 29610176
Malignant atrophic papulosis.
Scheinfeld N
Clin Exp Dermatol 2007 Sep;32(5):483-7. doi: 10.1111/j.1365-2230.2007.02497.x. PMID: 17692056
Malignant atrophic papulosis.
Degos R
Br J Dermatol 1979 Jan;100(1):21-35. doi: 10.1111/j.1365-2133.1979.tb03566.x. PMID: 371654

Clinical prediction guides

A loss-of-function NCSTN mutation associated with familial Dowling Degos disease and hidradenitis suppurativa.
de Oliveira ASLE, de Siqueira RC, Nait-Meddour C, Tricarico PM, Moura R, Agrelli A, d'Adamo AP, Jamain S, Crovella S, de Fátima Medeiros Brito M, Boniotto M, Brandão LAC
Exp Dermatol 2023 Nov;32(11):1935-1945. Epub 2023 Sep 4 doi: 10.1111/exd.14919. PMID: 37665193
Clinical and laboratory prognosticators of atrophic papulosis (Degos disease): a systematic review.
Lu JD, Sachdeva M, Silverberg OM, Shapiro L, Croitoru D, Levy R
Orphanet J Rare Dis 2021 May 6;16(1):203. doi: 10.1186/s13023-021-01819-z. PMID: 33957947Free PMC Article
The effects of Eculizumab on the pathology of malignant atrophic papulosis.
Magro CM, Wang X, Garrett-Bakelman F, Laurence J, Shapiro LS, DeSancho MT
Orphanet J Rare Dis 2013 Nov 26;8:185. doi: 10.1186/1750-1172-8-185. PMID: 24279613Free PMC Article
Possible involvement of SDF-1/CXCL12 in the pathogenesis of Degos disease.
Meephansan J, Komine M, Hosoda S, Tsuda H, Karakawa M, Murata S, Demitsu T, Ohtsuki M
J Am Acad Dermatol 2013 Jan;68(1):138-43. Epub 2012 Aug 27 doi: 10.1016/j.jaad.2012.06.031. PMID: 22951280
Optic nerve and spinal cord manifestations of malignant atrophic papulosis (Degos disease).
Matsuura F, Makino K, Fukushima T, Matsubara N, Shibuya M, Higuchi T, Hashidate H, Yamada M, Shibuya H, Yamazaki M
J Neurol Neurosurg Psychiatry 2006 Feb;77(2):260-2. doi: 10.1136/jnnp.2005.066134. PMID: 16421135Free PMC Article

Recent systematic reviews

Clinical and laboratory prognosticators of atrophic papulosis (Degos disease): a systematic review.
Lu JD, Sachdeva M, Silverberg OM, Shapiro L, Croitoru D, Levy R
Orphanet J Rare Dis 2021 May 6;16(1):203. doi: 10.1186/s13023-021-01819-z. PMID: 33957947Free PMC Article
Clinical manifestations and treatment outcomes in degos disease: a systematic review.
Kim PJ, Lytvyn Y, Kashetsky N, Bagit A, Mufti A, Yeung J
J Eur Acad Dermatol Venereol 2021 Aug;35(8):1655-1669. Epub 2021 May 16 doi: 10.1111/jdv.17311. PMID: 33914972

Supplemental Content

Table of contents

    Clinical resources

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • OMIM
      Related records in OMIM
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed
    • PubMed (OMIM)
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...