Term
| what is osler-weber rendu disease? |
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Definition
| osler-weber rendu disease or hereditary hemorrhagic telangiectasia causes bleeding in the nose, mouth, small intestine and may not present w/a mass. |
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Term
| what can blue rubber bleb nevi tell you? |
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Definition
| lesions that appear as moles, but are angiomas filled with blood and collapse on touch. these could be the cause of an occult GI bleed. |
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Term
| how does peutz-jeghers syndrome present? |
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Definition
| blue, red, brownish mucocutaneous lesions on the lips and other areas of mucosa associated with hamartomas (polypoid lesions in the GI tract that can bleed) |
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Term
| what is pseudoxanthoma elasticum? |
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Definition
| pts appear older than their age due to yellowed skin with decreased elasticity/tensile strength. looks like a "plucked chicken". due to rapid progression of calcification of elastic fiber, causing fragmentation |
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Term
| what is neurofibromatosis? |
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Definition
| the distribution of neurofibromas, which can appear on the skin, but also in the GI - where they can cause bleeds |
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Term
| what is ehlers-danlos syndrome? |
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Definition
| pts w/this have hypermobility of joints, hyperextensibility, hyperelasticity, and cigarette paper skin that is easily friable. can cause GI bleeds. |
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Term
| what is primary amyloidosis? |
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Definition
| abnormal deposition of amyloid which causes macroglossia (thick, large tongue) as well as fragmentation of capillaries in the skin w/a pinch test. |
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Term
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Definition
| a multiple idopathic hemorrhagic sarcoma which consists of *generally symmetrical blue, black, purple raised plaques which primarily affect the abdominal cavity. usually 2ndary to HIV. |
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Term
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Definition
| degos disease or *malignant atrophic papulosis consists of *palisading telangiectasias w/porcelain white centers that can cause erosions in the stomach, can progress in the intestine and perforate, causing peritonitis (can be fatal). |
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Term
| how do pts with scleroderma appear? what else needs to be considered in these pts? |
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Definition
| taut skin, pale, no hair on distal digits/knuckles, sores on fingertips (can cause bone demineralization due to lack of blood). vertical creases around the mouth. papillodema in eyes (bright light has rings). consider CREST syndrome (calcinosis, reynaud's, esophagitis, sclerodactyly, telangiectasia) |
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Term
| what is carcinoid syndrome? |
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Definition
| carcinoid tumors secrete serotonin, but it is bradykinin (powerful vasodilator) which causes pts to present as red, ruddy, and w/flushed complexion. the increased serotonin likely causes diarrhea. pellegra-like changes are seen in the hands, feet appear dusky (niacin deficiency). there may be fibrotic lesions in the heart and bronchoconstriction (wheezing). |
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Term
| what is acanthosis nigricans? |
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Definition
| hyperplastic, hyperpigmented, nonpruritic lesions in skin folds (often in the neck, appears "dirty"). there is an association with malignancy in the peritoneal cavity (mainly gastric CA). |
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Term
| can CA of the stomach cause bulli on the skin? |
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Definition
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Term
| what is dermatitis herpetiformis associated with? |
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Definition
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Term
| what is wilson's disease? |
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Definition
| Cu gets stuck in the liver and other tissues, leading to low serum ceruloplasm levels, and neurotic/pyschotic pt presentation. the kayser-fleischer ring in the cornea is diagnostic and tx: penicillimide. |
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