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20 Cards in this Set
- Front
- Back
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Difference between Raynaud's Disease and Raynaud's Phenomenon? |
Raynaud's Disease (primary)- benign vasoconstriction leading to the clinical presentation, self limited, no underlying disease
Raynaud's Phenomenon (secondary)- secondary to underlying disorder, usually a CT disease
**remember, blue is required |
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Just read this:
Raynaud's is multifactorial:
1. vascular pathogenesis 2. neural pathogenesis 3. intravascular pathogenesis |
1. vascular pathogenesis- structural --> severe fibrotic proliferation of the intima --> endothelial injury; endothelial dysfunction --> asymmetric NOS production
2. neural pathogenesis- emotional stress may precipitate attack, impaired vasodilation d/t decreased calcitonin gene related peptide and increased neuropeptide Y; increased vasoconstriction d/t increased alpha-2c adrenergic receptors in response to cold
3. intravascular pathogenesis- platelet aggregation and activation through thromboxane A2 (potent vasoconstrictor), elevated serotonin |
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Livedo reticularis is a common physiologic finding consisting of mottled reticulated vascular patter secondary to a physiologic vasospastic response mc due to cold --> alterations in flow through the microvasculature
What secondary symptoms can it be associated with? |
CTD Vasculitis Calciphylaxis Sneddon syndrome Livedo vasculopathy Hypercoagulable states Embolic states *** TQ |
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What is Sneddon syndrome? |
Arteriopathy characterized by stroke/TIA and livedo reticularis
Affects females in 30s and 40s, labile hypertension, history of fetal loss and Raynauds, some have APL, treat with warfarin |
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Before starting nifedipine for livedo reticularis/raynauds check... |
bp |
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Difference between livedo reticularis and racemosa? |
both livedo reticularis and racemosa due to decreased oxygenation and O2 tension in skin
reticularis- lacy network, connected, good prognosis
racemosa- broken lacy network, not connected, poor prognosis |
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What is Susac's syndrome? |
microangiopathy affecting capillaries of brain, retina and inner ear --> triad of encephalopathy, visual and hearing loss |
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What is Divry-Van Bogaert syndrome? |
A case of a progressive disease with epilepsy, marble skin, and roentgenographic evidence of tapering of the distal carotid branches with corticomeningeal angiomatosis was studied. The clinical course, angiographic findings, and skin biopsy results justified the diagnosis of noncalcifying venous capillary angiomatosis, or Divry-Van Bogaert syndrome. |
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What is CADASIL? Gene mutation? |
CADASIL ("Cerebral Autosomal-Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy") is the most common form of hereditary stroke disorder, and is thought to be caused by mutations of the Notch 3 gene on chromosome 19.
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What is carcinoid syndrome? Why is flushing significant? |
flushing, edema, bronchospasm, diarrhea, right sided CHF, peptic ulcers, hypotension
'carcinoid flush' in 10% of midgut tumors (small intestine, appendix and proximal colon) --> liver mets are required
type III gastric carcinoid are associated with pruritic, patch bright red flush
Bronchial tumors are associated with prolonged, intense red --> purple flush
hindgut tumors are rarely associated with flushing, even with liver mets |
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What is erythromelalgia? Types? |
erythromelalgia- paroxysmal vasodilation --> burning pain, redness, high skin temperature, lasts minutes to days, attacks happen late in the day or at night
1: associated with thrombocytopenia, can be unilateral and lead to necrosis
2: primary or idiopathic, many patients have SCN9A, more likely bilateral
3: secondary to d/o other than thrombocytopenia (myeloproliferative d/o, drugs- CCB, ergot derivatives) |
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Gain of function SCN9A? |
type 2 erythromelalgia --> peripheral sodium channel --> paroxysmal extreme pain disorder
** LOF causes loss of pain sensation |
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What is red ear syndrome? |
redness and burning pain of the ears, usually one at a time, attacks in the winter, a/w trigeminal neuralgia, migraines, SLE |
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What condition has gingival telangiectasias as an early extracutaneous manifestations? |
juvenile DM |
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Nevus anemicus? |
congenital pale area of skin with an irregular border d/t local blood vessels sensitive to endogenous catecholamines and permanent vasoconstricted
heat or ice accentuate the lesions |
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Mutations in Osler-Weber-Rendu? |
AD
HHT-1/HHT-2 encode endoglin and ALK-1 respectively
endoglin and ALK-1 are TGF binding proteins on vascular endothelium
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Findings in Osler-Weber-Rendu? |
recurrent epistaxis in children
mat like telangiectasias on MM in adolescence, cutaneous lesions post pubertal
GI involvement --> obvious hemorrhage or iron deficiency anemia
pulmonary AVM, paradoxical emboli
Tx: AVM embolization, octreotide for visceral hemorrhage, destruction to cutaneous lesions |
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Mutation in Ataxia-Telangiectasia? |
ATM gene encode phosphatidylinositol 3 kinase like serine/threonine protein kinase |
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Clinical features of Ataxia Telangiectasia? |
progressive cerebellar ataxia
oculocutaneous telangiectasias at 6 years old
progeric changes in skin and hair
sinopulmonary infections secondary to selective immunodeficiency
increased sensitivity to ionizing radiation
leukemia and lymphoma
elevated alpha-fetoprotein and CEA |
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Life span of ataxia telangiectasia? |
20-25 years |
