Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Dec 16, 2022; 10(35): 12971-12979
Published online Dec 16, 2022. doi: 10.12998/wjcc.v10.i35.12971
Malignant atrophic papulosis: Two case reports
Zhi-Gui Li, Jia-Ming Zhou, Li Li, Xiao-Dong Wang
Zhi-Gui Li, Li Li, Xiao-Dong Wang, Department of Gastrointestinal Surgery, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
Jia-Ming Zhou, Department of Clinical Medicine, West China School of Medicine, Sichuan University, Chengdu 610207, Sichuan Province, China
Author contributions: Li ZG and Zhou JM designed and wrote the manuscript; Li L and Wang XD reviewed the manuscript; all authors have read and approved the final manuscript.
Supported by Key Project of Science and Technology Department of Sichuan Province, No. 2022YFS0337.
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrollment.
Conflict-of-interest statement: All authors report having no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Xiao-Dong Wang, MD, Doctor, Department of Gastrointestinal Surgery, West China Hospital, Sichuan University, No. 37 Guoxue Lane, Wuhou District, Chengdu 610041, Sichuan Province, China. wangxiaodong@wchscu.cn
Received: June 22, 2022
Peer-review started: June 22, 2022
First decision: August 4, 2022
Revised: August 16, 2022
Accepted: November 18, 2022
Article in press: November 18, 2022
Published online: December 16, 2022
Abstract
BACKGROUND

Malignant atrophic papulosis is a rare and potentially lethal thrombo-occlusive microvasculopathy characterized by cutaneous papules and gastrointestinal perforation. The precise pathogenesis of this disease remains obscure.

CASE SUMMARY

We describe the case of a 67-year-old male patient who initially presented with cutaneous aubergine papules and dull pain in the epigastrium. One week after symptom onset, he was admitted to the hospital for worsening abdominal pain. Exploratory laparotomy showed patchy necrosis and subserosal white plaque lesions on the small intestinal wall, along with multiple perforations. Histological examination of the small intestine showed extensive hyperemia, edema, necrosis with varying degrees of inflammatory reactions in the small bowel wall, small vasculitis with fibrinoid necrosis and intraluminal thrombosis in the mesothelium. Based on the mentioned evidence, a diagnosis of malignant atrophic papulosis was made. We also present the case of a 46-year-old man with known cutaneous manifestations, abdominal pain, nausea and vomiting. His physical examination showed positive rebound tenderness. A computed tomography scan revealed free intraperitoneal air. He required surgical intervention on admission and then developed an esophageal perforation. He ultimately died of a massive hemorrhage.

CONCLUSION

In previously published cases of this disease, the cutaneous lesions initially appeared as small erythematous papules. Subsequently, the papules became porcelain-white atrophic depression lesions with a pink, telangiectatic peripheral rim. In one of the patients, the cutaneous lesions appeared as aubergine papules. The other patient developed multiple perforations in the gastrointestinal tract. Due to malignant atrophic papulosis affecting multiple organs, many authors speculated that it is not a specific entity. This case series serves as additional evidence for our hypothesis.

Keywords: Malignant atrophic papulosis, Gastrointestinal perforation, Papulosis, Thrombo-occlusive microvasculopathy, Case report

Core Tip: Malignant atrophic papulosis is a rare and potentially lethal thrombo-occlusive microvasculopathy characterized by cutaneous papules and gastrointestinal perforation.