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Fitzpatrick's Dermatology, 9th Edition (Chapter-based Decks) > 140: Erythema Elevatum Diutinum > Flashcards

140: Erythema Elevatum Diutinum Flashcards

1
Q

What are the clinical features of Erythema Elevatum Diutinum (EED)?

A

EED presents with erythematous to violaceous papules, nodules, and plaques, symmetrical distribution over joints and extensor surfaces, possible purpura, petechiae, vegetative lesions, ulcerations, and bullous appearance. Lesions are usually round to oval, smooth, and not fixed to underlying structures. They can be asymptomatic or associated with pruritus, pain, burning, stinging, paresthesia, or neuropathy. Systemic symptoms may include arthralgia and fever. Early lesions are often soft and tender, while older lesions may be firm or doughy. Lesions may coalesce into irregular patterns and can resemble xanthomata in color.

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2
Q

What are the two main types of Erythema Elevatum Diutinum (EED) and their characteristics?

A

The two main types of EED are Bury-type and Hutchinson-type.

Bury-type is more common in young women and often associated with personal and/or family history of rheumatism. Hutchinson-type is more common in older men and often associated with gout.

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3
Q

What is the pathogenesis of Erythema Elevatum Diutinum (EED)?

A

The pathogenesis of EED is not fully understood, but it is hypothesized that the formation of antigen-antibody complexes leads to deposition in blood vessel walls. Evidence supporting this includes induction of lesions with streptokinase injection, deposition of IgG, IgM, IgA, and fibrin in vascular and perivascular areas on immunofluorescence, and increased C1q-binding activity and enhanced IL-8 responses in affected patients’ sera.

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4
Q

What are the epidemiological characteristics of Erythema Elevatum Diutinum (EED)?

A

Epidemiological characteristics of EED include unknown incidence with only 250 cases reported. It can present at any age but is most common in the fourth to sixth decades of life. Rare cases have been reported in children. Historically, men are affected more often, but definitive sexual or ethnic predilection is difficult to ascertain due to the low number of cases.

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5
Q

What are the potential triggers for outbreaks of Erythema Elevatum Diutinum (EED)?

A

Potential triggers for outbreaks of EED include streptococcal infections and cold weather, which can exacerbate symptoms or lead to new lesions.

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6
Q

What is the likely diagnosis for a 45-year-old man with symmetric, violaceous plaques over his elbows and knees, worsened by winter?

A

The likely diagnosis is Erythema Elevatum Diutinum (EED). Cold weather exacerbates the symptoms or causes new lesions.

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7
Q

What condition should be considered in the differential diagnosis for a 60-year-old HIV-positive patient with lesions resembling Kaposi sarcoma?

A

Erythema Elevatum Diutinum (EED) should be considered because its varied appearance can resemble Kaposi sarcoma or bacillary angiomatosis in HIV-infected persons.

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8
Q

What subtype of EED is likely in a young woman with a family history of rheumatism and soft, erythematous papules over her joints?

A

The subtype is likely the ‘Bury-type,’ which is more often seen in young women with a personal and/or family history of rheumatism.

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9
Q

What subtype of EED is likely in an older man with a history of gout and firm, violaceous plaques on his extensor surfaces?

A

The subtype is likely the ‘Hutchinson-type,’ which is more often seen in older men with gout.

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10
Q

What does it suggest if a patient presents with erythematous plaques that are firm in the evening but softer in the morning?

A

This suggests Erythema Elevatum Diutinum (EED). The natural course varies, with spontaneous resolution after 5-10 years in some cases, while fixed lesions may last decades.

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11
Q

What does it suggest if a 50-year-old patient presents with lesions that coalesce into irregular patterns and develop a yellow hue?

A

This suggests Erythema Elevatum Diutinum (EED). The yellow hues resemble xanthomata.

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12
Q

What environmental factors might precipitate new outbreaks in a patient with EED?

A

Streptococcal infections and cold weather can precipitate outbreaks or exacerbate symptoms.

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13
Q

What areas are typically spared in a patient with EED who has lesions on the extensor surfaces of the elbows and knees?

A

The face, ears, buttocks, genitals, retroauricular area, palmoplantar skin, torso, and mucosal membranes are typically spared.

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14
Q

What other systemic symptoms might a patient with EED experience alongside fever and arthralgia?

A

Other systemic symptoms may include pruritus, pain, burning, stinging, paresthesia, or neuropathy.

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15
Q

What happens to lesions over time in a patient with EED who has firm and raised lesions in the evening?

A

Over time, the lesions coalesce into irregular patterns, become darker brown or violaceous, and may develop fibrosis.

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16
Q

What is the typical shape and surface of lesions in a patient with EED that are not fixed to underlying structures?

A

The lesions are round to oval, usually smooth, and without scale.

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17
Q

What is the typical color progression of lesions in a patient with EED that are erythematous in the evening?

A

The lesions progress from pink or erythematous to brown, violaceous, or yellow hues over time.

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18
Q

What does it suggest if a patient with EED has lesions that are tender and soft?

A

Tender and soft lesions suggest an early stage of EED.

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19
Q

What does it suggest if a patient with EED has lesions that are firm and doughy?

A

Firm and doughy lesions suggest an older stage of EED.

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20
Q

What hypothesis explains the pathogenesis of EED in a patient with lesions exacerbated by cold weather?

A

The hypothesis is that antigen-antibody complexes lead to deposition of immune complexes in blood vessel walls, causing the lesions.

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21
Q

What other immunologic findings support the pathogenesis hypothesis in a patient with EED who has increased C1q-binding activity?

A

Other findings include deposition of IgG, IgM, IgA, and fibrin in vascular and perivascular locations, and enhanced IL-8 responses.

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22
Q

What does it suggest if a patient with EED has lesions induced by streptokinase injection?

A

This suggests that streptokinase-streptodornase complexes may play a role in the formation of lesions, supporting the antigen-antibody complex hypothesis.

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23
Q

What clinical feature can help differentiate EED from Kaposi sarcoma in a patient with lesions resembling Kaposi sarcoma?

A

EED lesions are typically symmetric and distributed over joints and extensor surfaces, unlike Kaposi sarcoma.

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24
Q

What does it indicate if a patient with EED has lesions that are not fixed to underlying structures?

A

This indicates that the lesions are not invasive and are confined to the skin.

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25
Q

What systemic symptoms might accompany erythematous and raised lesions in a patient with EED?

A

Systemic symptoms may include fever, arthralgia, pruritus, pain, burning, stinging, paresthesia, or neuropathy.

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26
Q

What is Erythema Elevatum Diutinum (EED)?

A

A rare, chronic leukocytoclastic vasculitis characterized by symmetric papules and plaques, primarily over joints and extensor surfaces.

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27
Q

What are the two main types of Erythema Elevatum Diutinum?

A

Bury-type, more common in young women with a history of rheumatism, and Hutchinson-type, more common in older men with gout.

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28
Q

What is the typical age range for the presentation of Erythema Elevatum Diutinum?

A

Most common in the fourth through sixth decades of life, but can present at any age.

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29
Q

What are the clinical features of Erythema Elevatum Diutinum?

A

Erythematous to violaceous papules, nodules, and plaques, often symmetrical, primarily over joints and extensor surfaces.

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30
Q

What systemic symptoms may accompany Erythema Elevatum Diutinum?

A

Arthralgia and fever may be experienced with the eruption.

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31
Q

What is the proposed pathogenesis of Erythema Elevatum Diutinum?

A

Formation of antigen-antibody complexes leading to deposition of immune complexes in blood vessel walls.

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32
Q

What can precipitate outbreaks of Erythema Elevatum Diutinum?

A

Streptococcal infections and cold weather can exacerbate symptoms or cause new lesions.

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33
Q

How does the appearance of chronic Erythema Elevatum Diutinum vary in HIV-infected persons?

A

It can resemble Kaposi sarcoma or bacillary angiomatosis.

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34
Q

What is the natural course of Erythema Elevatum Diutinum?

A

It can vary; spontaneous resolution may occur after 5 to 10 years, while some lesions may last decades.

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35
Q

What are the clinical features of Erythema Elevatum Diutinum (EED) and how do they vary over time?

A

EED presents with edematous, erythematous to violaceous papules, nodules, and plaques that are symmetrically distributed over joints and extensor surfaces. Individual lesions are typically round to oval, smooth, and not fixed to underlying structures. Over time, lesions may coalesce into irregular patterns, darken in color, and may become firm or doughy. Symptoms can include pruritus, pain, and systemic symptoms like arthralgia and fever. The natural course varies, with some cases resolving spontaneously after 5 to 10 years, while others may persist for decades, with symptoms exacerbated by cold weather or streptococcal infections.

36
Q

How does the pathogenesis of Erythema Elevatum Diutinum (EED) relate to immune complex deposition?

A

The pathogenesis of EED is hypothesized to involve the formation of antigen-antibody complexes that lead to the deposition of immune complexes in blood vessel walls. Evidence supporting this includes induction of lesions with streptokinase injections, forming streptokinase-streptodornase complexes, direct immunofluorescence showing deposition of IgG, IgM, IgA, and fibrin in vascular and perivascular areas, and increased C1q-binding activity and enhanced IL-8 responses in the sera of affected patients.

37
Q

What are the differences between the Bury-type and Hutchinson-type of Erythema Elevatum Diutinum (EED)?

A

The Bury-type of EED is more commonly seen in young women with a personal or family history of rheumatism, while the Hutchinson-type is more frequently observed in older men with gout. Both types are now considered part of a spectrum of the disorder rather than distinct entities.

38
Q

What factors can precipitate outbreaks of Erythema Elevatum Diutinum (EED)?

A

Outbreaks of EED can be precipitated by streptococcal infections and may be exacerbated by cold weather, which can lead to the emergence of new lesions or worsening of existing symptoms.

39
Q

What is the epidemiology of Erythema Elevatum Diutinum (EED) regarding age and gender prevalence?

A

EED can present at any age but is most common in the fourth to sixth decades of life. Although men are affected more often, the low number of reported cases makes it difficult to determine any definitive sexual or ethnic predilection. The incidence remains unknown, with only 250 cases reported.

40
Q

What are the clinical features of Erythema Elevatum Diutinum (EED) and how do they vary over time?

A

Erythema Elevatum Diutinum (EED) presents with erythematous to violaceous papules, nodules, and plaques, symmetrical distribution over joints and extensor surfaces, purpura, petechiae, vegetative lesions, ulcerations, and bullous appearance may occur. Lesions are round to oval, smooth, and not fixed to underlying structures. Asymptomatic or associated with symptoms like pruritus, pain, burning, stinging, paresthesia, or neuropathy. Systemic symptoms such as arthralgia and fever may accompany eruptions. Early lesions are soft and tender, while older lesions may become firm or doughy. Over time, lesions may coalesce into irregular patterns, darken, and may exhibit fibrosis or yellow hues resembling xanthomata. The natural course of EED can vary, with spontaneous resolution occurring after 5 to 10 years, while in some cases, lesions may persist for decades.

41
Q

What is the proposed pathogenesis of Erythema Elevatum Diutinum (EED) and what evidence supports this hypothesis?

A

The pathogenesis of Erythema Elevatum Diutinum (EED) is not fully understood, but a hypothesis suggests that it involves the formation of antigen-antibody complexes leading to the deposition of immune complexes in blood vessel walls. Evidence supporting this includes induction of lesions with injection of streptokinase, leading to the formation of streptokinase-streptodornase complexes, deposition of immunoglobulins (IgG, IgM, IgA) and fibrin in vascular and perivascular locations observed through direct immunofluorescence examination, and increased C1q-binding activity and enhanced IL-8 responses in the sera of affected patients.

42
Q

How does the presentation of Erythema Elevatum Diutinum differ between the Bury-type and Hutchinson-type?

A

The presentation of Erythema Elevatum Diutinum (EED) varies between the two types: Bury-type is more often seen in young women with a personal and/or family history of rheumatism, while Hutchinson-type is more often seen in older men with a history of gout. Both types share common clinical features of EED, but their demographic associations differ significantly.

43
Q

What is the most frequent associated condition with Erythema Elevatum Diutinum (EED)?

A

The most frequent association with EED is IgA paraproteinemia. However, the severity of EED does not appear to depend on total paraprotein levels.

44
Q

Which type of EED is more often seen in young women with a personal and/or family history of rheumatism?

A

Bury-type EED.

45
Q

Which type of EED is more often seen in older men with gout?

A

Hutchinson-type EED.

46
Q

What is the most frequent associated condition with Erythema Elevatum Diutinum (EED)?

A

IgA paraproteinemia.

47
Q

What are the key histologic findings in the diagnosis of EED?

A

Key histologic findings in EED include:

  • Leukocytoclastic vasculitis as a central feature.
  • Early EED shows neutrophilic infiltrates and neutrophilic pyknotic debris.
  • Fibrin surrounding the upper and mid-dermal vascular plexi.
  • Late EED may show progressive fibrosis and sclerotic changes, with possible intracellular lipid deposition and rare cholesterol clefts.
48
Q

What are the first-line agents for the management of EED?

A

First-line agents for the management of EED include sulfone-based therapies, specifically dapsone and sulfapyridine. Rapid improvement may occur within the first 48 hours, with complete resolution over weeks or months.

49
Q

What is a notable histologic differential diagnosis for EED?

A

A notable histologic differential diagnosis for EED is Granuloma Faciale. Key differences include:

  • Granuloma Faciale is more likely to show admixed plasma cells and eosinophils, while EED shows histiocytes and granulomatous areas.
50
Q

What are the goals of therapy in managing EED?

A

The goals of therapy in managing EED include:

  1. Symptomatic relief
  2. Clearing of lesions
  3. Appropriate management of associated conditions
51
Q

What histologic feature is central to the disease process in EED?

A

Leukocytoclastic vasculitis is a central feature of the disease process in EED.

52
Q

What findings are expected in the early stages of EED?

A

Early EED often demonstrates neutrophilic infiltrates, neutrophilic pyknotic debris, and fibrin surrounding the upper and middermal vascular plexii.

53
Q

What findings are expected in the late stages of EED?

A

Late EED is progressively more fibrotic and possibly sclerotic. Intracellular lipid deposition and rare cholesterol clefts may be seen, especially in admixed histiocytes.

54
Q

What diagnostic method is used to confirm EED suspicion?

A

A deep punch skin biopsy is used, but there are no laboratory studies to confirm the diagnosis.

55
Q

How does IgA paraproteinemia affect the severity of EED?

A

The severity of EED does not appear dependent on total paraprotein levels, even though IgA paraproteinemia is the most frequent association.

56
Q

What additional therapy should be considered for HIV-positive patients with EED?

A

Antiretroviral therapy should be added to sulfone-based therapy in HIV-positive patients with EED.

57
Q

What alternative treatment can be considered for a patient with EED who develops anemia from dapsone?

A

Prednisone may be helpful in recalcitrant cases or when faced with dapsone-associated anemia.

58
Q

What treatment options are effective for localized EED with limited lesions?

A

Intralesional corticosteroids or high-potency topical corticosteroids can be effective in limited disease.

59
Q

What additional anecdotal therapies can be considered for EED unresponsive to first-line treatments?

A

Additional therapies include niacinamide, tetracyclines, colchicine, NSAIDs (e.g., diclofenac), chloroquine, phenformin, clofazimine, and cyclophosphamide.

60
Q

What rare disorder should be considered in the differential diagnosis of EED?

A

Granuloma Faciale should be considered, which is more likely to demonstrate admixed plasma cells and eosinophils.

61
Q

What histologic feature can help differentiate EED from Kaposi sarcoma?

A

Leukocytoclastic vasculitis with concentric perivascular fibrosis in later lesions is a distinguishing histologic feature of EED.

62
Q

What findings are typically observed in direct immunofluorescence studies for EED?

A

Direct immunofluorescence studies often reveal non-specific deposition of IgG, IgM, IgA, C3, and fibrin in perivascular locations.

63
Q

How quickly can improvement be expected with sulfone-based therapy for EED?

A

Rapid improvement may occur within the first 48 hours, with complete resolution of all lesions over weeks or months.

64
Q

What histologic feature might explain the xanthomata-like appearance in EED?

A

Intracellular lipid deposition and rare cholesterol clefts, especially in admixed histiocytes, might explain the xanthomata-like appearance.

65
Q

What term has been proposed to represent a late-stage common endpoint of granuloma faciale and EED?

A

‘Localized chronic fibrosing vasculitis’ has been proposed to represent a late-stage common endpoint.

66
Q

What histologic feature might be observed in firm and violaceous lesions of EED?

A

Leukocytoclastic vasculitis with concentric perivascular fibrosis might be observed.

67
Q

What is the long-term prognosis for EED lesions that are resistant to treatment?

A

Even with appropriate treatment, EED may remain nonresponsive or recur at various points across a lifetime.

68
Q

What first-line therapy is recommended for firm and erythematous lesions in EED?

A

Sulfone-based therapies, including dapsone and sulfapyridine, are recommended as first-line treatment.

69
Q

What is the most frequent association with Erythema Elevatum Diutinum (EED)?

A

IgA paraproteinemia.

70
Q

What is a central histologic feature of EED?

A

Leukocytoclastic vasculitis.

71
Q

What type of skin biopsy is used for diagnosing EED?

A

Deep punch skin biopsy, full thickness of the dermis extending into the upper subcutis.

72
Q

What histologic findings are seen in early EED?

A

Neutrophilic infiltrates and neutrophilic pyknotic debris, as well as fibrin surrounding the upper and middermal vascular plexii.

73
Q

What changes occur in late EED histologically?

A

Progressively more fibrotic and perhaps even sclerotic, with intracellular lipid deposition and rare cholesterol clefts.

74
Q

What is the goal of therapy for EED?

A

Symptomatic relief, clearing of lesions, and appropriate management of associated conditions.

75
Q

What are the first-line agents for treating EED?

A

Sulfone-based therapies, including dapsone and sulfapyridine.

76
Q

What additional therapies may be considered for EED?

A

Niacinamide, tetracyclines, colchicine, nonsteroidal anti-inflammatory drugs, chloroquine, phenformin, clofazimine, and cyclophosphamide.

77
Q

What should be added to sulfone-based therapy in HIV-positive patients with EED?

A

Antiretroviral therapy.

78
Q

What may improve the symptoms of EED related to associated conditions?

A

Treatment of underlying paraproteinemias.

79
Q

What is the most frequent associated condition with Erythema Elevatum Diutinum (EED) and how does it relate to the severity of EED?

A

The most frequent associated condition with EED is IgA paraproteinemia. However, the severity of EED does not appear to be dependent on total paraprotein levels.

80
Q

What are the key histologic findings in early and late stages of Erythema Elevatum Diutinum (EED)?

A

Early EED histologic findings include:
- Neutrophilic infiltrates
- Neutrophilic pyknotic debris
- Fibrin surrounding the upper and middermal vascular plexii

Late EED findings may show:
- Progressive fibrosis and possible sclerosis
- Intracellular lipid deposition
- Rare cholesterol clefts, especially in admixed histiocytes.

81
Q

What are the differential diagnoses to consider for Erythema Elevatum Diutinum (EED) in HIV-positive patients?

A

In HIV-positive patients, EED can mimic various stages of Kaposi sarcoma (patch, plaque, or nodular disease) and bacillary angiomatosis. Additionally, Granuloma Faciale should be considered in the histologic differential diagnosis.

82
Q

What are the first-line treatment options for Erythema Elevatum Diutinum (EED) and their expected outcomes?

A

The first-line treatment options for EED include sulfone-based therapies, such as dapsone and sulfapyridine. These treatments can lead to rapid improvement within the first 48 hours, with complete resolution of lesions occurring over weeks or months.

83
Q

How does the management of underlying paraproteinemias affect the symptoms of Erythema Elevatum Diutinum (EED)?

A

The treatment of underlying paraproteinemias improves the symptoms of EED.

84
Q

What is a close histologic differential diagnosis of EED?

A

A close histologic differential diagnosis of EED is cutaneous leukocytoclastic vasculitis.

85
Q

Which areas of the body are typically spared in EED?

A

The face and trunk are typically spared in EED.

86
Q

True or False: There are no laboratory studies to confirm a diagnosis of EED.

A

True.

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

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