Malignant Atrophic Papulosis Presenting with Intestinal Perforation: A Case Report.
Journal
JNMA; journal of the Nepal Medical Association
ISSN: 1815-672X
Titre abrégé: JNMA J Nepal Med Assoc
Pays: Nepal
ID NLM: 0045233
Informations de publication
Date de publication:
01 Jun 2023
01 Jun 2023
Historique:
received:
24
05
2023
medline:
21
7
2023
pubmed:
19
7
2023
entrez:
19
7
2023
Statut:
epublish
Résumé
Malignant atrophic papulosis sometimes known as Degos' disease is an idiopathic, uncommon condition with fewer than 200 occurrences documented. It is a chronic thrombo-obliterative vasculopathy characterised by papular skin lesions with a core porcelain-white atrophy and a surrounding telangiectatic border. We report a 15-year-old male patient with a recurrent history of hollow viscus perforation, which was managed on all the occasions with exploratory laparotomy and primary perforation repair. Additionally, the patient had a five month history of numerous, non-itchy, atrophic papules with a core porcelain-like area and hyperkeratotic margins, characteristic of Degos' disease. The only basis for diagnosis is the distinctive skin lesions with biopsy. Along with systemic lupus erythematosus and other connective tissue diseases, tuberculosis must also be taken into account while assessing the clinical presentation of malignant atrophic papulosis. There is currently no known treatment for malignant atrophic papulosis that has been effective. case reports; intestinal perforation; malignant atrophic papulosis; ulcer; vasculitis.
Identifiants
pubmed: 37464847
doi: 10.31729/jnma.8192
pmc: PMC10276933
doi:
Substances chimiques
Dental Porcelain
12001-21-7
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
549-551Références
Int J Dermatol. 2000 May;39(5):361-2
pubmed: 10849127
Br J Dermatol. 2014 Jan;170(1):110-5
pubmed: 24116834
Arch Dermatol. 1963 Jan;87:54-62
pubmed: 14001179
Clin Exp Dermatol. 2007 Sep;32(5):483-7
pubmed: 17692056
Obstet Med. 2016 Dec;9(4):167-168
pubmed: 27829877