[Köhlmeier-Degos disease (Malignant Atrophic Papulosis): a cause of recurrent multiple intestinal perforations]. 2009

M Beuran, and Al L Chiotoroiu, and S Morteanu, and A Chilie, and M Avram, and O Roşu, and M Vartic, and C Creangă, and A Mangrău
Clinica de Chirurgie, Spitalul Clinic de Urgenţă Bucureşti. drmirceabeuran@yahoo.com

Malignant Atrophic Papulosis (MAP) or Degos disease is a very rare vasculopathy affecting multiple systems. When associated with gastrointestinal tract or central nervous system involvment, patients with Degos disease have a poor prognosis and a high mortality. We report a case of Degos disease with systemic involvement, which ultimately caused peritonitis, sepsis, and death, despite all treatment. A 29-year-old man was admitted in June 2007 on the surgical ward of our hospital with acute generalized abdominal pain and multiple skin lesions. The patient had an appendectomy at another hospital 6 weeks before the current presentation. The exploratory laparotomy showed 2 perforations of the ileum. After the initial procedure, the patient developed spontaneous recurrent perforations of the small bowel and suffered another 3 reoperations. Neuroradiologic reports described central and peripheral nervous system involvement with progressive clinical deterioration and a meningovascular pattern at cerebral MRI. Despite aggressive treatment the patient died 3 months after the first surgical intervention.

UI MeSH Term Description Entries
D007416 Intestinal Perforation Opening or penetration through the wall of the INTESTINES. Intestinal Perforations,Perforation, Intestinal,Perforations, Intestinal
D008297 Male Males
D010538 Peritonitis INFLAMMATION of the PERITONEUM lining the ABDOMINAL CAVITY as the result of infectious, autoimmune, or chemical processes. Primary peritonitis is due to infection of the PERITONEAL CAVITY via hematogenous or lymphatic spread and without intra-abdominal source. Secondary peritonitis arises from the ABDOMINAL CAVITY itself through RUPTURE or ABSCESS of intra-abdominal organs. Primary Peritonitis,Secondary Peritonitis,Peritonitis, Primary,Peritonitis, Secondary
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D015746 Abdominal Pain Sensation of discomfort, distress, or agony in the abdominal region. Colicky Pain,Abdominal Pains,Colicky Pains,Pain, Abdominal,Pain, Colicky,Pains, Abdominal,Pains, Colicky
D054853 Malignant Atrophic Papulosis Variously described as a vasculopathy, endovasculitis, or occlusive arteriopathy, this condition occurs in a benign cutaneous form and a lethal multiorgan systemic variant. It is characterized by a narrowing and occlusion of the lumen of small to medium-sized blood vessels, leading to ischemia and infarction in the involved organ systems. The etiology and pathophysiology are unknown. Degos Disease,Kohlmeier-Degos Disease,Degos Syndrome,Degos's Malignant Atrophic Papulosis,Erythrokeratoderma en cocardes,Papulosis, Malignant Atrophic,Atrophic Papuloses, Malignant,Atrophic Papulosis, Malignant,Disease, Degos,Disease, Kohlmeier-Degos,Malignant Atrophic Papuloses,Papuloses, Malignant Atrophic,Syndrome, Degos
D017809 Fatal Outcome Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept. Fatal Outcomes,Outcome, Fatal,Outcomes, Fatal
D018805 Sepsis Systemic inflammatory response syndrome with a proven or suspected infectious etiology. When sepsis is associated with organ dysfunction distant from the site of infection, it is called severe sepsis. When sepsis is accompanied by HYPOTENSION despite adequate fluid infusion, it is called SEPTIC SHOCK. Bloodstream Infection,Pyaemia,Pyemia,Pyohemia,Blood Poisoning,Poisoning, Blood,Septicemia,Severe Sepsis,Blood Poisonings,Bloodstream Infections,Infection, Bloodstream,Poisonings, Blood,Pyaemias,Pyemias,Pyohemias,Sepsis, Severe,Septicemias

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