​When abdominal pain is combined with this kind of pimples, the 3-year survival rate is less than 50%...

Only for medical professionals to read for reference.
Small pimples, but kill them? The Chinese case that climbed to the top of Gut In March this year, a case report by Professor Liang Jie, deputy chief physician of the Department of Gastroenterology, Xijing Hospital of the Air Force Medical University of China, was a case report entitled unusual cause of abdominal pain and papule (unusual cause of abdominal pain and papule) Top journal Gut in the digestive field (impact factor: 19.
819)
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Once the case was released, it quickly aroused widespread attention and discussion among gastroenterology clinicians internationally
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Figure 1: The case report published by Professor Liang Jie's team on Gut is published in an authoritative journal.
What are the highlights of this case? In the case, the patient had multiple abdominal papules for more than 9 months, accompanied by intermittent melena and abdominal pain for more than 5 months.
The abdominal pain worsened for 3 days and was sent to the Gastroenterology Department of Xijing Hospital for diagnosis and treatment, and was handled by Professor Liang Jie
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Under the diagnosis of Professor Liang Jie's team, the true face of the disease was revealed-Degos disease (malignant atrophic papulosis), which is a rare necrotizing vasculitis
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The disease can be divided into pure skin type and systemic manifestation with skin manifestation.
Systemic Degos disease progresses rapidly, and the gastrointestinal tract is one of its main involved parts
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Moreover, as a fatal disease, most patients with Degos disease die from intestinal perforation and intestinal necrosis, which is inextricably linked with the digestive system and also brings great challenges to the diagnosis and treatment of gastroenterologists
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Unfortunately, because the disease is extremely dangerous and there is no specific treatment, the patient in this case died shortly after refusing a second operation
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The medical community is fortunate to invite Professor Liang Jie, the corresponding author of this article, to start from this case, to further introduce and share the overall understanding of Degos disease in China and the current status of diagnosis and treatment, and to provide some suggestions for patients with Degos disease
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But before that, let’s take a look at the “truth” of this case and see what tests Professor Liang Jie used in the diagnosis process, and how to diagnose this disease at first glance, which seems to be incompatible with the digestive system disease.
And" diseases
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Case overview ▌ History of present illness: The elderly have multiple abdominal papules (approximately 2-15mm in size) for more than 9 months, accompanied by intermittent melena, and abdominal pain for 5 months
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Three days before admission, the patient's abdominal pain worsened
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Figure 2: Multiple papules on the abdomen of the elderly▌ Pre-hospital examination: Colonoscopy: Chronic inflammation of the colonic mucosa (pathological results show active mucosal inflammation, positive for Epstein-Barr virus)
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Figure 3: Chronic inflammation of the colonic mucosa can be seen under colonoscopy.
CT enterography: stenosis of the ileum in the right lower abdomen
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▌ Inspection and treatment during hospitalization: C-reactive protein: 31.
53 ng/L, fibrinogen: 5.
77 g/L, D-dimer: 1308 ng/ml
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On the day of hospitalization, the patient's sudden abdominal pain aggravated and stopped gas and defecation
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Physical examination: abdominal tenderness, rebound pain
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Abdominal X-ray: suggest intestinal obstruction
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Emergency laparotomy.
During the operation, it was found that the abdomen was filled with a lot of gas, yellow feces and abscesses, and the small intestine was frequently ischemic and perforated (7 locations)
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▌ Postoperative examination and diagnosis: Postoperative pathological examination of the small bowel: lymphocyte necrosis, swelling, degeneration, and necrosis of collagen fibers in the ischemic area, ulcers and infarctions of the intestinal wall
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Figure 4: Pathological examination of the skin biopsy of the lesion area after small bowel surgery: leukocytosis vasculitis, small artery occlusion, epidermal atrophy, dermal collagen fiber disorder
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Figure 5: After comprehensive evaluation of skin biopsy of the lesion area, it was diagnosed as Degos disease
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After the case was published, the editor immediately contacted Professor Liang Jie, the author of the article, and asked Professor Liang Jie to answer some of the questions most concerned about Degos disease clinicians, and shared his own diagnosis and treatment ideas.
The highlights are here
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What is Degos disease, and how lack of understanding of it by Chinese gastroenterologists? Professor Liang Jie said that Degos disease is a rare vascular disease that affects the inner walls of middle and small arteries and veins, which can lead to vascular blockage and tissue infarction
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The disease is not a tumor, but the prognosis is extremely poor and the fatality is extremely high
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It is documented that the 3-year survival rate of Degos disease patients with systemic disease is less than 50%, which brings an extremely heavy burden to individuals and their families
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Degos disease is more common in middle-aged and young (20-40 years old) men.
The lesions usually involve the skin (often the first manifestation) and intestines (about 50% of patients)
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The disease can also affect the central nervous system, even the eyes, heart, kidneys, and bladder, but it is less common
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Its etiology is not yet clear.
The current academic view is that it may be related to factors such as autosomal dominant inheritance, autoimmune abnormalities, decreased fibrinolytic activity, and chronic viral infections
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From a macro perspective, China's gastroenterologists generally lack knowledge of Degos disease.
Doctors in major gastroenterology departments are likely to be in a state of "unheard of, unheard of"
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However, doctors at the Inflammatory Bowel Disease (IBD) center generally have a certain degree of understanding of the disease
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What signs should be alert to Degos disease? Professor Liang Jie believes that for patients with rash and gastrointestinal symptoms, clinicians should consider the possibility of different system manifestations of the same disease, and avoid treating the rash and gastrointestinal symptoms as two isolated symptoms
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The most important thing is to respond quickly and combine it with infectious bowel disease, IBD or other rheumatic immune system-related bowel diseases, such as Behcet’s disease, polyarteritis nodosa, purpura, etc.
, and even tumor diseases such as lymphoma, Gastrointestinal tumors, gastrointestinal stromal tumors and other diseases for differential diagnosis
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According to literature records, the typical skin lesions of Degos disease are pale rose-colored round edematous papules with a diameter of 2-5 mm, gradually expanding and depressed in the center of the papules, which are umbilical fossa-like.
Cyan red with dilated capillaries, and finally atrophy
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The skin lesions occur in batches, are scattered, and rarely merge with each other.
They generally have no symptoms, can last for months and years, and are often misdiagnosed as psoriasis
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Therefore, if a gastroenterologist finds ulcers and inflammatory lesions under colonoscopy, and notices that the patient has the above-mentioned characteristic rash, he should be alert to Degos disease
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The final diagnosis depends on the pathological biopsy results of the diseased intestines and skin lesions
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What is the current standard treatment plan for Degos disease in my country? There is no exact and effective treatment for Degos disease
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But in the final analysis, it is a kind of small vasculitis.
Like many other vasculitis in the Department of Rheumatology and Immunology, the basic pathological changes are arteritis and thrombosis
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For vascular problems, anticoagulation and antiplatelet therapy are generally used clinically, combined with hormone therapy, and immunosuppressive agents can also be used
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If the patient has digestive ulcers, salicylic acid preparations should be added
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If intestinal obstruction or perforation occurs, emergency surgery should be performed
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What can a gastroenterologist do to maximize the prognosis? As mentioned earlier, the overall prognosis of the disease is extremely poor, and because the number of cases is very small, Professor Liang Jie mentioned that what can be done to help improve the prognosis, and even whether the prognosis can be improved is still unknown
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However, Professor Liang Jie also firmly believes that for gastroenterologists, early recognition of Degos disease is still very important, which has a certain effect on delaying the development of the disease, and at least can reduce the risk of rapid perforation in a short time
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How should various disciplines work together to improve the diagnosis and treatment of Degos disease in my country? The concept of multidisciplinary joint diagnosis and treatment was founded in the field of oncology, but Professor Liang Jie believes that it is also very suitable for bowel diseases
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Patients with Degos disease have clear skin lesions.
If there is no rash, it is not enough to diagnose Degos disease
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This means that dermatologists need to further diagnose and differentially diagnose the nature of the rash
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In addition, because skin lesions generally have no symptoms, patients are likely to see intestinal symptoms, which can easily be misdiagnosed by gastroenterologists as other digestive diseases, such as IBD accompanied by skin lesions
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When intestinal perforation occurs, if the surgeon lacks knowledge of the disease and ignores the connection between skin lesions and intestinal lesions, it is easy to misdiagnose it as simple intestinal perforation
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The diagnosis of this disease often requires pathological examinations of intestinal lesions and skin lesions at the same time.
It can be seen that at least the cooperation of gastroenterology, endoscopy center, surgery, dermatology, and pathologists is involved
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To make a clear diagnosis in time, doctors in these disciplines must have a full understanding of Degos disease
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Can you give some advice to patients with Degos disease? Degos disease has no obvious cause and is difficult to prevent
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However, if the patient has already developed characteristic rash and gastrointestinal symptoms that cannot be explained by conventional diseases, and the condition still deteriorates after initial treatment, or the remission is not satisfactory, if conditions permit, it is recommended to go to a large bowel disease center for further diagnosis and treatment as soon as possible.
It is not a stay in a general digestive clinic or a skin clinic
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Due to the lack of knowledge of the disease in ordinary hospitals, if only symptomatic treatment of the digestive tract or skin symptoms is performed alone, it is likely to delay the disease and miss the opportunity for early corrective treatment
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 Expert profile: Professor Liang Jie, Professor, Ph.
D.
, post-doctoral supervisor of Xijing Hospital of Gastroenterology, Virginia Commonwealth University, USA Postdoctoral Fellow, Cleveland Medical College, USA Visiting Scholar, Chinese Digestive Inflammatory Bowel Disease Group Secretary and Member, Cross-Strait Medicine and Health Exchange Association Digestive Committee Youth Committee Chairman, Chinese Association of Integrated Traditional Chinese and Western Medicine, Digestive Endoscopy Professional Committee, Vice Chairman of Intestinal Microecology Expert Committee, Permanent Member of the Youth Committee of China Anti-Cancer Association, Vice Chairman of Shaanxi Gastroenterology Society, Specialty: Good at inflammatory bowel disease, digestion Department of tumor, gastroesophageal reflux disease, functional gastrointestinal disease and other digestive diseases, clinical diagnosis and treatment and basic research
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Published more than 70 SCI papers and won the first prize of National Science and Technology Progress Award in 2008; 100 national outstanding doctoral theses in 2010; China Youth Science and Technology Award in 2013; Outstanding Youth Fund of the National Fund Committee in 2013; National Innovation Team Award in 2016; 2016 Young and middle-aged innovation leaders of the Ministry of Science and Technology; 2018 National Ten Thousand Talents Plan; 2019 Young Changjiang Scholars of the Ministry of Education
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References: [1] Zhou H, Chen F, Wang J, et al.
Unusual cause of abdominal pain and papuleGut Published Online First: 08 March 2021.
doi:10.
1136/gutjnl-2021-324270.
[2] Wang Xianfa, Huang Diyu, Xinying et al.
Analysis and evaluation of difficult cases[J].
The 98th case of avascular necrosis of the greater omentum—small bowel perforation—skin rash—Degos disease.
Chinese Medical Journal.
2006(6).
[3]Xue Hongqian, Xu Dongkui, Zhang Jiefeng, etc.
.
Degos disease combined with ileal perforation in 1 case[J].
Chinese Journal of Gastrointestinal Surgery.
2000(3).
[4],.
Analysis of 2 cases of misdiagnosis of Degos disease and literature review[J].
Clinical misdiagnosis and mistreatment.
2010 (10).