Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033377 (prolapse)
 11,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 49-year-old woman with typical skin lesions of Degos' disease was found to have an afferent pupillary defect, and altitudinal field loss. This loss was probably secondary to vascular occlusion in the optic nerve of a patient with a congenital anomalous retinal vascular pattern. The patient has had no other systemic involvement after a follow-up of 4 years. Three other patients seen in the past had eye involvement and involvement of the central nervous system and died between 1 and 16 years of the diagnosis. Multiple ocular manifestations of Degos' disease have been reported affecting the eyelids, bulbar conjunctivae, retina, and choroid in varying degrees. Diplopia, visual field defects, ophthalmoplegia, ptosis, papilledema, and optic atrophy are ocular changes secondary to involvement of the central nervous system.
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PMID:Ophthalmic changes of Degos' disease (malignant atrophic papulosis). 671 15

We present the case of a 57-year-old woman with a 1-year history of a cutaneous eruption clinically and histopathologically characteristic of Degos disease. In one of the two cutaneous biopsy specimens taken from the trunk lesions, the histopathology consisted of necrosis and sclerosis of the subcutaneous lobules, a finding that to our knowledge has not been previously described in the cutaneous lesions of Degos disease. Two months after cutaneous biopsies were taken, the patient developed ptosis and an episode of acute abdominal pain; intestinal perforation and many characteristic lesions of visceral Degos disease involving the entire small bowel were noted at laparotomy.
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PMID:Panniculitis mimicking lupus erythematosus profundus: a new histopathologic finding in malignant atrophic papulosis (Degos disease). 1044 79

Degos disease, or malignant atrophic papulosis, is a rare vasculopathy of uncertain aetiology manifesting as a primary dermatological disorder in most cases, but with widespread systemic involvement developing in an undefined proportion of patients. Reported neurological features of Degos disease include ischaemic and haemorrhagic stroke, subdural effusion, seizures, neuropathy, transverse myelitis, and optic atrophy. The description of contrast enhancement of the leptomeninges possibly indicates a defect of blood vessel integrity likely explaining the pleiotropic neurological manifestations. Degos disease is usually considered a disorder of adulthood, although a small number of infantile cases have been described. Here, we report a female who demonstrated a neonatal onset of Degos disease, eventually showing the highly characteristic skin lesions together with ptosis and a generalized weakness as part of her systemic disorder. Subsequent exacerbations led to an inexorable neurodevelopmental and physical decline. CT scan revealed intracranial calcification, a feature described in two previous cases. Our report highlights the need to consider Degos disease in the differential diagnosis of childhood neurological disease with skin involvement.
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PMID:Infantile neurological Degos disease. 2065 9