Malignant atrophic papulosis (Degos disease).

Malignant atrophic papulosis (Degos disease) is a rare syndrome of multiple-system vascular diseases with unknown etiology. It can affect the skin, gastrointestinal tract and central nervous system. Here, we report a 58-year-old woman with extensive porcelain-white atrophic papules. Based on the clinical manifestations, skin biopsy and colonoscopy, a diagnosis of malignant atrophic papulosis was confirmed.

[Case of disseminated intestinal and cutaneous thromboangiitis (Kohlmeier-Degos' syndrome)].

Disseminated intestinal and cutaneous thromboangiitis (Kohlmeier-Degos' syndrome) is a rare syndrome of unknown etiology, which is characterized by cutaneous and visceral involvement. Two (cutaneous and visceral) stages are identified. A fatal outcome is inevitable although there may be a long (8-20-year) course of the disease during a benign process. The causes of death are peritonitis, central nervous system impairments, pleurisy, pericarditis. The paper describes a case of disseminated intestinal and cutaneous thromboangiitis (Kohlmeier-Degos' syndrome) in a 32-year-old female patient who has died from intoxication due to severely impaired permeability of the epithelial barrier mainly of the large bowel (diphtheritic inflammation of the mucosa, severe inflammatory edema of the submucous layer, and fibrinous impregnation of the serous membrane).

[Degos' disease: A case report and review of literature].

A 18-year-old male patient's case was diagnosed as Degos'disease with pathognomonic skin lesions, accompanied by small bowel perforation, and reported here. Skin histopothological test show that the typical wedge-shaped necrobiosis and lymphocyte inflammatory infiltration. Vessels showed narrowing and thrombosis, with lymphocyte infiltration. Degos' disease is a systemic necrotizing vasculitis. Skin biopsy can confirm its diagnosis. Severe systemic complication should be prevented. Degos' disease should be considered in the differential diagnosis of skin lesions associated with systemic involvement.

Degos disease: a new simulator of non-accidental injury.

Recent high-profile cases have made paediatricians very aware of the serious implications of either missing or wrongly diagnosing non-accidental injury. Subdural fluid collections in non-mobile infants usually represent haemorrhage caused by non-accidental injury. We report a 6-month-old male who presented to the Accident and Emergency Department of Birmingham Heartlands Hospital with bilateral subdural fluid collections and skin ulcers resembling cigarette burns. Non-accidental injury was considered to be the most likely diagnosis. However, while under observation in hospital, the child's neurological condition deteriorated with progressive cerebral infarctions, and serial photographs of the skin lesions showed failure to heal. The revised diagnosis, confirmed histologically, was Degos disease, an extremely rare and often fatal occlusive vasculopathy. The child was treated palliatively and died 8 weeks after presentation. This report informs doctors of a new simulator of non-accidental injury to be considered in infants with otherwise unexplained subdural fluid collections.

Small bowel infarcts in Degos disease.

Degos disease is a rare idiopathic vasculopathy with multisystem involvement. We present a previously healthy 40-year-old woman who developed spontaneous small bowel infarctions and perforations 17 months after presentation with characteristic skin lesions. The abdominal CT findings of Degos disease, which have not been previously depicted in the radiology literature, are discussed.