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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.
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Gastrointestinal hemorrhage
MedGen UID:
8971
•Concept ID:
C0017181
•
Pathologic Function
Hemorrhage affecting the gastrointestinal tract.
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Gastrointestinal infarctions
MedGen UID:
463581
•Concept ID:
C3152231
•
Finding
See: Feature record | Search on this feature
Pleural effusion
MedGen UID:
10805
•Concept ID:
C0032227
•
Disease or Syndrome
The presence of an excessive amount of fluid in the pleural cavity.
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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.
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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
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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

The color of skin: white diseases of the skin, nails, and mucosa.
Brown AE, Qiu CC, Drozd B, Sklover LR, Vickers CM, Hsu S
Clin Dermatol 2019 Sep-Oct;37(5):561-579. Epub 2019 Jul 17 doi: 10.1016/j.clindermatol.2019.07.018. PMID: 31896410
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
Degos' syndrome complicated by bowel perforation: focus on radiological findings.
Yeung JT, Ma JK, Yung AW
Hong Kong Med J 2013 Apr;19(2):174-7. PMID: 23535679
Images in dermatology. The red flag.
Wobser M, Burger M, Trautmann A
Am J Med 2010 Jan;123(1):31-3. doi: 10.1016/j.amjmed.2009.09.016. PMID: 20102988
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
Cutaneous manifestations of antiphospholipid syndrome.
Gomez-Flores M, Herrera-Argaez G, Vazquez-Martinez O, Herz-Ruelas M, Ocampo-Candiani J, Villarreal-Martinez A, Galarza-Delgado DA, Hernandez-Galarza I, Chavez-Alvarez S
Lupus 2021 Apr;30(4):541-548. Epub 2021 Feb 14 doi: 10.1177/0961203321990100. PMID: 33583236
Challenging mimickers of primary systemic vasculitis.
Miloslavsky EM, Stone JH, Unizony SH
Rheum Dis Clin North Am 2015;41(1):141-60, ix. doi: 10.1016/j.rdc.2014.09.011. PMID: 25399945
Case for diagnosis.
Lima Lde A, Haddad Nde C, Lima RB, D'Acri AM, Martins CJ
An Bras Dermatol 2014 May-Jun;89(3):521-2. doi: 10.1590/abd1806-4841.20142863. PMID: 24937836Free PMC Article
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

Diffused Degos disease treated with rivaroxaban.
Gu X, Jiang X, Chen M
Ital J Dermatol Venerol 2022 Jun;157(3):294-295. doi: 10.23736/S2784-8671.21.07088-2. PMID: 35707869
Degos disease complicated by constrictive pericarditis in remote phase: a case report.
Tadokoro Y, Kitamura T, Horai T, Miyaji K
J Cardiothorac Surg 2022 Apr 1;17(1):59. doi: 10.1186/s13019-022-01810-0. PMID: 35365165Free PMC Article
Clinical and Histopathologic Characteristics of the Main Causes of Vascular Occusion - Part II: Coagulation Disorders, Emboli, and Other.
Beato Merino MJ, Diago A, Fernandez-Flores A, Fraga J, García Herrera A, Garrido M, Idoate Gastearena MA, Llamas-Velasco M, Monteagudo C, Onrubia J, Pérez-González YC, Pérez Muñoz N, Ríos-Martín JJ, Ríos-Viñuela E, Rodríguez Peralto JL, Rozas Muñoz E, Sanmartín O, Santonja C, Santos-Briz A, Saus C, Suárez Peñaranda JM, Velasco Benito V
Actas Dermosifiliogr (Engl Ed) 2021 Feb;112(2):103-117. Epub 2020 Oct 16 doi: 10.1016/j.ad.2020.10.004. PMID: 33075291
Worsening skin lesions but no diagnosis.
Lubsen J
J Fam Pract 2020 Dec;69(10):E11-E13. PMID: 33348351
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

Prognosis

Atrophic Papulosis.
Robert M, Hot A
Dermatology 2023;239(2):177-187. Epub 2022 Nov 9 doi: 10.1159/000526789. PMID: 36353773
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
Case for diagnosis.
Lima Lde A, Haddad Nde C, Lima RB, D'Acri AM, Martins CJ
An Bras Dermatol 2014 May-Jun;89(3):521-2. doi: 10.1590/abd1806-4841.20142863. PMID: 24937836Free PMC Article
Infantile neurological Degos disease.
Yeo TH, Vassallo G, Judge M, Laycock N, Kelsey A, Crow YJ
Eur J Paediatr Neurol 2011 Mar;15(2):167-70. Epub 2010 Jul 24 doi: 10.1016/j.ejpn.2010.06.003. PMID: 20659809
Cutaneous manifestations of antiphospholipid antibody syndrome.
Weinstein S, Piette W
Hematol Oncol Clin North Am 2008 Feb;22(1):67-77, vi. doi: 10.1016/j.hoc.2007.10.011. PMID: 18207066

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
Degos disease: a new simulator of non-accidental injury.
Moss C, Wassmer E, Debelle G, Hackett S, Goodyear H, Malcomson R, Ryder C, Sgouros S, Shahidullah H
Dev Med Child Neurol 2009 Aug;51(8):647-50. Epub 2009 Jun 22 doi: 10.1111/j.1469-8749.2009.03349.x. PMID: 19549203
Benign Degos' disease developing during pregnancy and followed for 10 years.
Bogenrieder T, Kuske M, Landthaler M, Stolz W
Acta Derm Venereol 2002;82(4):284-7. doi: 10.1080/000155502320323261. PMID: 12361134

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

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