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Fitzpatrick's Dermatology, 9th Edition (Chapter-based Decks) > 145: Raynaud Phenomenon > Flashcards

145: Raynaud Phenomenon Flashcards

1
Q

What are the two forms of Raynaud Phenomenon (RP)?

A

Primary (idiopathic) RP and Secondary RP.

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2
Q

How does Primary Raynaud Phenomenon differ from Secondary Raynaud Phenomenon?

A

Primary RP is an exaggerated physiologic response to cold or emotional stimuli and does not result in tissue injury. Secondary RP occurs due to a systemic disorder or drug exposure, leading to more aggressive tissue ischemia.

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3
Q

What is the prevalence of Raynaud Phenomenon in the general population?

A

Up to 10%.

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4
Q

What is the prevalence of Raynaud Phenomenon among patients with systemic sclerosis?

A

90%.

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5
Q

What demographic factors are associated with Raynaud Phenomenon?

A

Strong female predominance with a 4:1 female-to-male ratio, symptoms often develop during the second decade of life, increased frequency and severity during menstrual cycles, and associations with cold climate, cardiovascular disease, low body mass index, and use of vibratory tools.

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6
Q

What are the clinical features and symptoms of Raynaud Phenomenon?

A

Patients experience episodic attacks of white or blue digits due to cold exposure and emotional stimuli, affected digits may be numb and turn bright red upon rewarming, episodes usually last for about 30 minutes but can persist for hours.

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7
Q

What triggers the attacks in patients with Raynaud phenomenon?

A

Cold exposure and emotional stimuli.

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8
Q

What is a common symptom during an attack of Raynaud phenomenon?

A

Affected digits become numb and may turn bright red upon rewarming.

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9
Q

What is the typical duration of episodes of Raynaud phenomenon?

A

Usually lasts for 30 minutes but can persist for hours.

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10
Q

What does secondary Raynaud phenomenon indicate?

A

It occurs as a result of a systemic disorder or drug exposure and can lead to more severe tissue ischemia.

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11
Q

What are the cutaneous findings associated with Raynaud Phenomenon (RP)?

A

Well-demarcated pallor or cyanosis, cold and pale skin distal to the line of ischemia, cyanotic skin upon rewarming, trophic changes, atrophy, dystrophic nail changes, alopecia, true sclerodactyly, and ulcerations.

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12
Q

What is the significance of the Allen test in evaluating Raynaud Phenomenon?

A

The Allen test assesses arterial and capillary function. Abnormal filling implies structural disease of the microcirculation, raising suspicion for secondary RP.

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13
Q

What are the implications of secondary Raynaud Phenomenon in terms of clinical management?

A

Secondary RP is associated with more severe symptoms and potential complications, necessitating identification and treatment of the underlying disorder.

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14
Q

What symptoms suggest a connective tissue disorder in patients with Raynaud Phenomenon (RP)?

A

Arthralgias, dysphagia, muscle weakness, photosensitivity, gastroesophageal reflux, shortness of breath, or sicca symptoms.

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15
Q

What does persistent ischemic discoloration after rewarming suggest in Raynaud Phenomenon?

A

It suggests secondary disease.

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16
Q

What are trophic changes of the digits a sign of in Raynaud Phenomenon?

A

They are signs of prolonged attacks of RP and point to secondary disease.

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17
Q

What does true sclerodactyly indicate in a patient with Raynaud Phenomenon?

A

It warrants a high degree of suspicion for systemic sclerosis (SSc).

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18
Q

What is the significance of abnormal capillaries in the proximal nailfold for patients with Raynaud Phenomenon?

A

They portend a 60-fold increased risk for progression to systemic sclerosis (SSc).

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19
Q

What is the progression rate of primary Raynaud Phenomenon (RP) to secondary forms associated with connective tissue diseases during the first decade of onset?

A

As high as 15%.

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20
Q

What features are predictive of the progression from primary to secondary Raynaud Phenomenon?

A

Nailfold capillary abnormalities, hand edema, positive Allen’s test, and antinuclear antibodies.

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21
Q

What percentage of patients with systemic sclerosis (SSc) manifest symptoms of Raynaud Phenomenon?

A

80% to 90%.

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22
Q

In which connective tissue diseases is Raynaud Phenomenon commonly observed?

A

Systemic lupus erythematosus, autoimmune myositis, and systemic vasculitis.

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23
Q

What are some neurological disorders that may cause Raynaud Phenomenon?

A

Carpal tunnel syndrome, reflex sympathetic dystrophy, and nerve root compression.

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24
Q

What is the primary cause of Primary Raynaud Phenomenon (RP)?

A

It develops without an identifiable systemic or drug cause.

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25
Q

What can arteriograms of patients with connective tissue diseases show?

A

Digital and sometimes ulnar or radial artery obstructions.

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26
Q

What is a prominent feature of reflex sympathetic dystrophy that can simulate Raynaud Phenomenon?

A

Thermoregulatory changes.

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27
Q

What occupational exposures may contribute to the development of Raynaud Phenomenon?

A

The use of vibratory tools or perpetual hand trauma.

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28
Q

What are the predictive features for the progression of Primary Raynaud Phenomenon to a secondary form?

A

Predictive features include: Nailfold capillary abnormalities, Hand edema (puffy hands), Positive Allen’s test, Antinuclear antibodies.

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29
Q

What is the significance of Raynaud Phenomenon in patients with systemic sclerosis (SSc)?

A

In patients with systemic sclerosis (SSc): 80% to 90% manifest symptoms of Raynaud Phenomenon. It is the presenting sign in approximately one-third of patients and may be the only manifestation of the disease for years.

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30
Q

How does Raynaud Phenomenon present in patients with systemic lupus erythematosus and other connective tissue diseases?

A

In patients with systemic lupus erythematosus and other connective tissue diseases: Raynaud Phenomenon develops in up to a third of patients. It may be the only symptom for years, indicating the need for careful monitoring.

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31
Q

What are the potential neurological causes of Raynaud Phenomenon?

A

Neurological causes of Raynaud Phenomenon include: Thermoregulatory changes from reflex sympathetic dystrophy, Nerve root compression, Complications from carpal tunnel syndrome.

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32
Q

What role do drugs play in the development of Raynaud Phenomenon?

A

Certain drugs are associated with Raynaud Phenomenon, including: β-Adrenergic blockers, Ergotamines, Oral contraceptives, Methylsergide, Bleomycin, Vinblastine, Cyclophosphamide, Amphetamines, Interferon-α and -β, Imatinib.

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33
Q

What occupational factors increase the risk of developing Raynaud Phenomenon (RP)?

A

Individuals who work with vibratory tools (e.g., air hammers, chainsaws, rivet guns) and those exposed to cold temperatures (e.g., butchers, ice cream workers, fish packers) are at risk for RP.

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34
Q

What medications are commonly associated with causing Raynaud Phenomenon (RP)?

A

Common medications that can cause RP include: β-Adrenergic Blockers (e.g., Propranolol), Vasoconstrictive Drugs (e.g., Clonidine, ergot alkaloids), Chemotherapy Agents (e.g., Bleomycin, cisplatin), Other (e.g., Interferon, sulfasalazine).

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35
Q

What are the vascular abnormalities associated with Raynaud Phenomenon (RP)?

A

In Raynaud Phenomenon, endothelial dependent vasodilation is reduced, particularly in primary and SSc-related RP. Inadequate production of vasodilatory mediators and excessive vasoconstriction are critical.

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36
Q

What is the etiology and pathogenesis of Raynaud Phenomenon (RP)?

A

The etiology of Raynaud Phenomenon involves complex interactions between the vasculature, the nervous system, and circulating cytokines.

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37
Q

A 28-year-old patient with RP reports using vibratory tools at work. What occupational factors could contribute to their condition?

A

Occupational factors such as working with vibratory tools or prolonged exposure to cold temperatures can contribute to RP.

38
Q

A patient with RP is found to have cryoglobulins in their blood. What does this indicate?

A

The presence of cryoglobulins indicates hyperviscosity, which can contribute to the development of RP.

39
Q

What is the most common cause of iatrogenic Raynaud Phenomenon?

A

β-Adrenergic blockers, particularly propranolol, are the most common cause of iatrogenic RP.

40
Q

Which medications are known to cause Raynaud Phenomenon through vasoconstrictive mechanisms?

A

Clonidine, ergot alkaloids, dopaminergic agonists, and centrally acting stimulants are known to cause RP by vasoconstrictive mechanisms.

41
Q

What is a known side effect of treatment with interferon related to Raynaud Phenomenon?

A

RP can occur as a side effect of treatment with interferon, with some cases requiring surgical amputation due to ischemic injury.

42
Q

What role do endothelial dependent vasodilation and vasoconstriction play in Raynaud Phenomenon?

A

Reduced endothelial dependent vasodilation and excessive vasoconstriction contribute to the symptoms of RP.

43
Q

What are some miscellaneous disorders that can lead to the development of Raynaud Phenomenon?

A

Patients with hyperviscosity from cryoglobulins, macroglobulins, and polycythemia can develop RP.

44
Q

What are the key vascular abnormalities associated with Raynaud Phenomenon (RP)?

A

Key vascular abnormalities in RP include: Impaired vasodilation and enhanced vasoconstriction due to endothelin-1 receptor activity, Microangiopathy and intimal wall hyperplasia.

45
Q

What neural abnormalities are observed in patients with SSc-associated Raynaud Phenomenon?

A

Neural abnormalities in SSc-associated RP include: Reduced calcitonin gene-related peptide in sensory neurons, Alteration of the neurovascular axis, Central mechanisms that may trigger attacks due to emotional stress.

46
Q

What intravascular abnormalities are commonly seen in Raynaud Phenomenon?

A

Common intravascular abnormalities in RP include: Platelet activation, increased thrombin generation, Defective fibrinolysis, and increased viscosity.

47
Q

What are the diagnostic criteria for primary Raynaud Phenomenon?

A

To diagnose primary RP, patients must exhibit: Unusual cold sensitivity, Biphasic color changes during vasospastic episodes, and at least 3 specific features.

48
Q

What other mechanisms may contribute to the development of primary Raynaud Phenomenon?

A

Other mechanisms contributing to primary RP include hormonal influences from estrogen and genetic predisposition.

49
Q

A 35-year-old patient presents with episodic attacks of white and blue discoloration in their fingers triggered by cold exposure. What diagnostic criteria would you use to determine if this is primary Raynaud Phenomenon (RP)?

A

To diagnose primary RP, the patient must have unusual cold sensitivity, biphasic color changes, and meet at least 3 specific criteria.

50
Q

A patient with RP is found to have elevated levels of endothelin-1. What role does this molecule play in RP pathogenesis?

A

Endothelin-1 contributes to excessive vasoconstriction and acral hypoperfusion in RP.

51
Q

What role does the endothelin-1 receptor play in SSc-associated Raynaud’s phenomenon (RP)?

A

Inhibition of the endothelin-1 receptor with bosentan attenuates symptoms of SSc-associated RP.

52
Q

What are the structural changes associated with secondary Raynaud’s phenomenon?

A

Microangiopathy and intimal wall hyperplasia characterize the structural changes.

53
Q

How do neural abnormalities contribute to Raynaud’s phenomenon?

A

Reduced immunoreactive sensory neurons in the skin of fingers contribute to defective vasodilation.

54
Q

What central mechanisms may trigger attacks in Raynaud’s phenomenon?

A

Attacks can be elicited by emotional stress.

55
Q

What intravascular abnormalities are seen in Raynaud’s phenomenon?

A

Platelet activation, increased thrombin generation, defective fibrinolysis, and increased viscosity are among the intravascular alterations seen in RP.

56
Q

What hormonal influences are associated with primary Raynaud’s phenomenon?

A

Hormonal influences from estrogen and genetic predisposition have the strongest data supporting an etiologic association with primary RP.

57
Q

What genetic factors may contribute to susceptibility to primary Raynaud’s phenomenon?

A

Polymorphisms in genes encoding portions of the neuromuscular acetylcholine receptor and serotonin receptors may confer genetic susceptibility.

58
Q

What are the criteria for diagnosing Primary Raynaud Phenomenon?

A

The criteria for Primary Raynaud Phenomenon include: Vasospastic attacks, Bilateral involvement, Normal vascular examination, Absence of underlying disease, Negative antinuclear antibody test.

59
Q

What are some features that can support a diagnosis of Raynaud’s phenomenon?

A

Features include attacks accompanied by numbness, involvement of both hands, and well-demarcated color changes between affected and unaffected skin.

60
Q

What are the key vascular abnormalities associated with Raynaud Phenomenon (RP) and how do they contribute to the condition?

A

Key vascular abnormalities in RP include: Impaired vasodilation and enhanced vasoconstriction due to endothelin-1, Microangiopathy and intimal wall hyperplasia.

61
Q

How do neural abnormalities contribute to the pathophysiology of Raynaud Phenomenon?

A

Neural abnormalities in RP include reduced calcitonin gene-related peptide in sensory neurons and central mechanisms that can trigger attacks.

62
Q

What are the criteria for Primary Raynaud Phenomenon?

A

The criteria include vasospastic attacks, bilateral involvement, normal vascular examination, absence of underlying disease, negative antinuclear antibody test, and history of symptoms for at least 2 years.

63
Q

What laboratory studies should be conducted for patients suspected of having Raynaud Phenomenon?

A

Laboratory studies include complete blood count, erythrocyte sedimentation rate, urinalysis, and antinuclear antibody testing.

64
Q

What special testing can help predict the progression of Raynaud Phenomenon to systemic sclerosis (SSc)?

A

Nailfold capillaroscopy and thermal imaging can help predict progression to SSc.

65
Q

What does the presence of giant capillaries and capillary dropout on nailfold microscopy indicate?

A

These findings are highly specific for and can predict progression to systemic sclerosis (SSc).

66
Q

What are the triphasic color changes associated with Raynaud Phenomenon?

A

The triphasic color changes are white, blue, and red.

67
Q

At what age is secondary Raynaud Phenomenon more likely to occur?

A

Age at onset of 30 years or older.

68
Q

What clinical signs suggest a connective tissue disease in Raynaud Phenomenon?

A

Signs include arthritis or abnormal lung function.

69
Q

What is a significant finding in nailfold microscopy for secondary Raynaud Phenomenon?

A

Evidence of microvascular disease, particularly capillary dropout and giant capillary loops.

70
Q

What is the purpose of thermal imaging in Raynaud Phenomenon?

A

To measure digital and acral surface temperatures and distinguish between primary RP and those with SSc.

71
Q

What should be tested in patients with abnormal antinuclear antibody titers?

A

Testing for antibodies to specific nuclear antigens, such as centromere or topoisomerase.

72
Q

What are the criteria for diagnosing Primary Raynaud Phenomenon based on episodes and color changes?

A

Criteria include episodes at other body sites, triphasic color changes, bilateral involvement, normal vascular examination, absence of underlying disease, negative antinuclear antibody test, and history of symptoms for at least 2 years.

73
Q

What laboratory studies should be obtained for Raynaud Phenomenon?

A

Complete blood count, erythrocyte sedimentation rate, urinalysis, and antinuclear antibody testing.

74
Q

What clinical features suggest a higher likelihood of Secondary Raynaud Phenomenon?

A

Features include age at onset of 30 years or older, intense or painful episodes, signs of connective tissue disease, presence of specific autoantibodies, and evidence of microvascular disease.

75
Q

How can nailfold capillaroscopy be utilized in the context of Raynaud Phenomenon?

A

It can predict progression to systemic sclerosis (SSc) and identify capillary dilation, focal hemorrhage, and areas of avascularity.

76
Q

What role does thermal imaging play in differentiating between primary Raynaud Phenomenon and secondary conditions?

A

It measures digital and acral surface temperatures to distinguish between primary RP and secondary conditions such as SSc.

77
Q

What is acrocyanosis and how does it differ from Raynaud Phenomenon?

A

Acrocyanosis is characterized by blue-violet discoloration of acral skin due to diminished oxyhemoglobin and shows persistent color changes regardless of cold exposure.

78
Q

What are the characteristics and symptoms of pernio?

A

Pernio is an acral inflammatory dermatosis due to cold, wet conditions, characterized by erythematous to violaceous macules, pain, pruritus, and burning sensation.

79
Q

What is erythromelalgia and how does it present differently from Raynaud Phenomenon?

A

Erythromelalgia is characterized by pain, burning, edema, and erythema in warm limbs, triggered by exercise or warm exposure.

80
Q

Describe livedo reticularis and its physiological basis.

A

Livedo reticularis is a mottled, net-like vascular pattern that blanches with pressure, resulting from alterations in blood flow through the cutaneous microvasculature.

81
Q

What is reflex sympathetic dystrophy and what are its key features?

A

Reflex sympathetic dystrophy is a painful disorder associated with vasomotor instability due to autonomic nervous system dysfunction, often arising after limb injury.

82
Q

What is acrocyanosis characterized by?

A

Blue-violet discoloration of acral skin due to diminished oxyhemoglobin.

83
Q

What triggers the development of pernio?

A

Exposure to cold, wet conditions.

84
Q

What are the symptoms of erythromelalgia?

A

Symptoms include pain, burning, edema, and erythema, most often of the extremities.

85
Q

How does livedo reticularis appear?

A

As a mottled, well-formed reticulated vascular pattern that blanches with pressure.

86
Q

What is reflex sympathetic dystrophy associated with?

A

Vasomotor instability resulting from autonomic nervous system dysfunction.

87
Q

What is a common complication of severe cases of pernio?

A

Hemorrhagic lesions, bullae, loss of nails, and ulcerations.

88
Q

What percentage of erythromelalgia cases involve the feet?

A

90%.

89
Q

What is the typical duration of lesions in pernio?

A

Approximately 1 to 3 weeks.

90
Q

What is the relationship between erythromelalgia and Raynaud Phenomenon?

A

They may exist on a spectrum, with RP representing excessive vasoconstriction and erythromelalgia representing exaggerated vasodilation.

91
Q

What are the key characteristics that differentiate acrocyanosis from Raynaud Phenomenon (RP)?

A

Acrocyanosis shows persistent blue-violet discoloration without triphasic color changes, while RP is characterized by episodic color changes in response to cold or stress.

Fitzpatrick's Dermatology, 9th Edition (Chapter-based Decks) (48 decks)

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