Publication: Malignant Atrophic Papulosis Presenting with Intestinal Perforation: A Case Report
Date
2023
Journal Title
Journal ISSN
Volume Title
Publisher
Nepal Medical Association
Abstract
Abstract
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.
Description
Asmita Paudel
Department of Internal Medicine, Pokhara Academy of Health Sciences, Ramghat, Pokhara, Nepal
Min Raj Bhurtel
Department of Internal Medicine, Manipal College of Medical Sciences, Fulbari, Pokhara, Nepal
Ashok Gautam
Department of Internal Medicine, Pokhara Academy of Health Sciences, Ramghat, Pokhara, Nepal
Amrit Gautam
Department of Infectious Disease, Sukraraj Tropical and Infectious Disease Hospital, Teku, Kathmandu, Nepal
Mamata Bista
Department of Infectious Disease, Sukraraj Tropical and Infectious Disease Hospital, Teku, Kathmandu, Nepal
Pragyat Singh
Department of Internal Medicine, Chhatrapati Free Clinic Hospital, Chhatrapati, Kathmandu, Nepal
Keywords
case reports, Intestinal perforation, Malignant atrophic papulosis, Ulcer, Vasculitis