Widespread keratinocytic epidermal nevus with an associated chylous pericardial effusion

Widespread keratinocytic epidermal nevus with an associated chylous pericardial effusion

A 17-year-old male presented for review of a widespread keratinocytic epidermal nevus (KEN) in the setting of a chronic pericardial effusion. Biopsy of the epidermal nevus revealed a KRAS mutation. Pericardiocentesis revealed a chylous effusion and magnetic resonance lymphangiogram demonstrated an u...

Full description

Saved in:
Bibliographic Details
Published in:Pediatric dermatology Vol. 40; no. 5; pp. 962 - 963
Main Authors: Rowe, Georgina, Snyder, Kristen M, Treat, James R
Format: Journal Article
Language:English
Published: United States Wiley Subscription Services, Inc 01-09-2023
Subjects:
Biopsy
Case reports
Effusion
Mutation
Nevus
Pediatrics
hamartomas
epidermal nevi
KRAS
genodermatoses
genetic diseases/mechanisms
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:A 17-year-old male presented for review of a widespread keratinocytic epidermal nevus (KEN) in the setting of a chronic pericardial effusion. Biopsy of the epidermal nevus revealed a KRAS mutation. Pericardiocentesis revealed a chylous effusion and magnetic resonance lymphangiogram demonstrated an underlying lymphatic malformation. There are rare case reports of KEN with an associated KRAS mutation. This case highlights the importance of being alert to epidermal nevus syndrome, particularly in patients with a widespread nevus and seemingly unrelated pathology.
Bibliography:ObjectType-Case Study-2
SourceType-Scholarly Journals-1
ObjectType-Feature-4
content type line 23
ObjectType-Report-1
ObjectType-Article-3
ISSN:0736-8046
1525-1470
DOI:10.1111/pde.15323