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Fitzpatrick's Dermatology, 9th Edition (Entrance Exam / Board Exam Prep) > 146: Degos Disease > Flashcards

146: Degos Disease Flashcards

1
Q

What are the characteristic clinical features of malignant atrophic papulosis (Degos disease)?

A

Malignant atrophic papulosis is characterized by numerous, typical, porcelain-white atrophic papules with a rim of rosy erythema and/or telangiectasia. The lesions start as crops of 2-10mm, asymptomatic to mildly pruritic, rose-colored macules that progress to round, smooth dome-shaped firm papules, some with central umbilication and/or necrosis, evolving into porcelain-white atrophic papules within days to weeks.

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2
Q

What is the epidemiology of malignant atrophic papulosis (Degos disease)?

A

Malignant atrophic papulosis is rare, with approximately 250 cases reported in the literature. It almost always occurs in whites, with cases also observed in African Americans and Japan. The disease most commonly presents during the third to fourth decades of life and can occur at any age, with a male-to-female ratio of 3:1. The majority of cases are sporadic, but familial cases have been reported with an autosomal dominant pattern.

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3
Q

What are the major pathologic mechanisms involved in Degos disease?

A

The major pathologic mechanisms in Degos disease include vascular coagulopathy and/or endothelial cell damage. Several disease processes converge to produce clinical and histopathologic findings, including a combination of prothrombotic factors, platelet aggravation via CXCR4 receptor, and interferon-α-mediated endotheliopathy syndrome.

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4
Q

What are the differences between classic Degos disease and benign Degos disease?

A

Feature | Classic Degos Disease | Benign Degos Disease |
|———|———————|———————|
| Involvement | Can involve skin and other organs | Only skin involvement |
| Lesion Count | Varies from few to more than 100 | Typically fewer lesions |
| Systemic Symptoms | Usually precede systemic symptoms (e.g., GI or CNS issues) | No systemic symptoms reported |
| Familial Cases | Some familial cases reported | Most familial cases are benign |

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5
Q

What conditions are associated with secondary Degos disease-like lesions?

A

Secondary Degos disease-like lesions occur in patients with lupus erythematosus, dermatomyositis, systemic sclerosis, granulomatosis with polyangiitis, Crohn disease, and opioid-induced thrombotic microangiopathy.

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6
Q

What is the most likely diagnosis for porcelain-white atrophic papules with rosy erythema?

A

The most likely diagnosis is Degos disease. It can occur as idiopathic disease (classic Degos disease or its benign variant) or as a surrogate clinical finding in connective tissue diseases such as antiphospholipid syndrome, lupus erythematosus, dermatomyositis, or systemic sclerosis.

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7
Q

What is the typical progression of lesions in Degos disease?

A

The lesions start as rose-colored macules, progress to dome-shaped firm papules with central umbilication or necrosis, and evolve into porcelain-white atrophic papules with a rim of rosy erythema. Fully developed lesions resemble atrophie blanche.

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8
Q

What are the three major organ systems affected in classic Degos disease?

A

The three major organ systems affected are the skin, gastrointestinal (GI) system, and central nervous system (CNS). Systemic symptoms include bowel perforation (GI) and hemorrhagic or ischemic stroke (CNS).

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9
Q

What does the presence of antiphospholipid antibodies suggest in Degos disease?

A

The presence of antiphospholipid antibodies suggests a possible relationship between Degos disease and primary antiphospholipid antibody syndrome (APAS).

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10
Q

What are the clinical differences between classic Degos disease and benign Degos disease?

A

Classic Degos disease involves systemic symptoms (GI and CNS involvement) and a higher risk of death, while benign Degos disease is limited to skin involvement and is often familial with a better prognosis.

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11
Q

What is the most common ocular manifestation of Degos disease?

A

The most common ocular manifestation is an avascular patch on the conjunctiva.

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12
Q

What is the major pathologic mechanism underlying Degos disease?

A

The major pathologic mechanism is vascular coagulopathy and/or endothelial cell damage, leading to thrombotic microangiopathy.

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13
Q

What is the role of complement-mediated injury in Degos disease?

A

Complement-mediated injury, involving the membranolytic complement attack complex C5b-9, is a probable late event in Degos disease. This explains why eculizumab, an anti-C5 antibody, can rescue acutely ill patients.

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14
Q

What is the significance of a linear distribution of lesions in Degos disease?

A

A linear distribution of lesions has been reported but is not typical. Exclusive acral localization suggests a connective tissue disease rather than Degos disease.

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15
Q

What is the role of interferon-α in the pathogenesis of Degos disease?

A

Interferon-α is implicated in an endotheliopathy syndrome, contributing to thrombogenic microangiopathy through the membranolytic complement attack complex C5b-9.

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16
Q

What is the typical site of cutaneous involvement in Degos disease?

A

The typical sites of cutaneous involvement are the trunk and limbs. The palms, soles, face, scalp, and genitalia are usually spared.

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17
Q

What is the significance of antiphospholipid antibodies in Degos disease?

A

Antiphospholipid antibodies are seen in some patients with Degos disease, raising the possibility of a relationship with primary antiphospholipid antibody syndrome (APAS).

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18
Q

What is the significance of familial cases of Degos disease?

A

Familial cases of Degos disease are often benign and follow an autosomal dominant pattern.

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19
Q

What is the relationship between Degos disease and connective tissue diseases?

A

Degos disease can occur as a surrogate clinical finding in connective tissue diseases such as lupus erythematosus, dermatomyositis, systemic sclerosis, and antiphospholipid syndrome.

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20
Q

What is the clinical significance of atrophie blanche-like lesions in Degos disease?

A

Atrophie blanche-like lesions are a hallmark of Degos disease and may also occur at sites of interferon injection, indicating drug-induced lesions.

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21
Q

What is Malignant Atrophic Papulosis (Degos Disease)?

A

A rare, primary vasoocclusive disorder affecting the skin, GI, and CNS.

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22
Q

What are the clinical characteristics of Degos Disease lesions?

A

They are characterized by numerous, typical, porcelain-white atrophic papules with a rim of rosy erythema and/or telangiectasia.

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23
Q

What is the major pathologic mechanism in Degos Disease?

A

Vascular coagulopathy and/or endothelial cell damage.

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24
Q

What is the typical age range for the presentation of Degos Disease?

A

Most commonly presents during the third to fourth decades of life, but can occur at any age.

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25
What is the gender distribution of Degos Disease cases?
Men are affected more than women, with a ratio of approximately 3:1.
26
What are the possible ocular manifestations of Degos Disease?
Avascular patches on the conjunctiva and possible involvement of the sclera, episclera, retina, choroids, and optic nerves.
27
What distinguishes classic Degos Disease from benign Degos Disease?
Classic Degos Disease can involve systemic symptoms and multiple lesions, while benign Degos Disease is limited to skin involvement.
28
What are some associated conditions with secondary Degos Disease?
Lupus erythematosus, dermatomyositis, systemic sclerosis, granulomatosis with polyangiitis, Crohn disease, and opioid-induced thrombotic microangiopathy.
29
What is the significance of antiphospholipid antibodies in Degos Disease?
They are seen in some patients and raise the possibility of a relationship with primary antiphospholipid antibody syndrome (APAS).
30
What are the key clinical features of malignant atrophic papulosis (Degos disease)?
- **Numerous, typical, porcelain-white atrophic papules** - **Rim of rosy erythema and/or telangiectasia** - Lesions evolve from rose-colored macules to dome-shaped firm papules with central umbilication and/or necrosis. - Fully developed lesions resemble atrophie blanche, leaving a varicelliform white scar over time.
31
What is the significance of vascular coagulopathy in the pathogenesis of Degos disease?
- Vascular coagulopathy and/or endothelial cell damage is a **major pathologic mechanism**. - It involves a combination of prothrombotic factors that may trigger full-blown disease. - Platelet aggravation via CXCR4 receptor and interferon-α-mediated endotheliopathy syndrome contribute to the disease process.
32
How do the clinical presentations of classic Degos disease differ from benign Degos disease?
| Feature | Classic Degos Disease | Benign Degos Disease | |---------|---------------------|---------------------| | Lesion Count | Varies from few to over 100 | Only skin involvement | | Extracutaneous Involvement | Possible (GI, CNS) | None | | Familial Cases | Rare | Most are familial | | Symptoms | Precede systemic symptoms (e.g., GI perforation, stroke) | Asymptomatic lesions only |
33
What are the potential complications associated with classic Degos disease?
- **Hemorrhagic or ischemic stroke** due to small-vessel thrombotic involvement. - **Bowel perforation** as a systemic symptom. - Possible involvement of other organs such as kidneys, bladder, and eyes, leading to further complications.
34
What are the common ocular manifestations in patients with Degos disease?
- Most common ocular manifestation is an **avascular patch on the conjunctiva**. - Other possible ocular involvements include **sclerae, episcleral tissue, retina, choroids, and optic nerves** which may also be affected.
35
What are the histopathological findings in fully developed lesions of Degos disease?
The histopathological findings include: 1. **Cell-poor, wedge-shaped area** of mild-to-severe dermal necrosis with dermal edema and copious mucin at the papillary dermis to deep reticular dermis. 2. **Occluded vessels** with occasional thrombosis, thickened vessel walls, and intimal fibrosis at the base of the lesion. 3. **Proliferating endothelial cells**. 4. **Sparse perivascular lymphocytic infiltrate**. Note: Full-blown leukocytoclastic, neutrophilic vasculitis is never found, hence it is not classified as vasculitis.
36
What are the major complications associated with Degos disease?
The most frequent complications and major ominous events include: 1. **GI hemorrhage** 2. **Perforation** 3. **Peritonitis** These complications occur in up to **73% of the 30%** of patients with the systemic form of the disease. Less common complications include neurologic issues such as hemiparesis, aphasia, and seizures.
37
What is the prognosis for patients with Degos disease who do not have extracutaneous manifestations within 7 years?
If no extracutaneous manifestations occur within 7 years, there is a probability of a **benign monosymptomatic cutaneous course** of greater than **90%**. Conversely, lethality with extracutaneous involvement is greater than **50%**, primarily due to severe intestinal involvement.
38
What are the first-line treatment options for Degos disease?
First-line treatment options for Degos disease include: - **Platelet aggregation inhibitors** such as: - Aspirin - Clopidogrel - Dipyridamole Other treatments may include antibiotics, immunosuppressives, and anticoagulation strategies, but their success varies.
39
What laboratory tests should be performed for suspected Degos disease?
Laboratory tests that should be performed include: - **ANA** (Antinuclear Antibodies) - **Lupus anticoagulant** - **Anticardiolipin antibodies** - **Antiphospholipid antibodies** - **Cryoglobulins** - Extensive exploration of hemostasis - If disease is sudden in onset, do PCR to look for **parvovirus B19 DNA**.
40
What histopathologic findings are characteristic of fully developed Degos disease lesions?
Histopathologic findings include a cell-poor, wedge-shaped area of dermal necrosis, dermal edema, copious mucin deposition, occluded vessels with occasional thrombosis, thickened vessel walls, intimal fibrosis, proliferating endothelial cells, and sparse perivascular lymphocytic infiltrate.
41
Why is Degos disease not classified as vasculitis despite some overlapping features?
Degos disease is not classified as vasculitis because full-blown leukocytoclastic or neutrophilic vasculitis is never found in its histopathology.
42
What laboratory tests should be performed to evaluate a patient suspected of having Degos disease?
Tests include ANA, lupus anticoagulant, anticardiolipin antibodies, antiphospholipid antibodies, cryoglobulins, and PCR for parvovirus B19 DNA if the disease onset is sudden. Imaging studies like cerebral MRI and endoscopic evaluation/laparoscopy are mandatory if there are signs of GI or CNS involvement.
43
What are the most frequent complications and major ominous events in Degos disease?
The most frequent complications are GI hemorrhage, perforation, and peritonitis. Neurologic complications like hemiparesis, aphasia, and seizures are less common. Death is often related to ischemic or GI events.
44
What is the 5-year survival rate for patients with systemic Degos disease, and what factors influence prognosis?
The 5-year survival rate for patients with systemic Degos disease is 54%. Prognosis is better if there is no extracutaneous involvement within 2 years of diagnosis or if the disease follows a benign monosymptomatic cutaneous course for at least 7 years.
45
What is the first-line treatment for classic Degos disease, and what are some alternative treatments?
The first-line treatment is platelet aggregation inhibitors such as aspirin, clopidogrel, or dipyridamole. Alternative treatments include antibiotics, chloroquine, immunosuppressives, fibrinolytics, and anticoagulation strategies like LMWH or newer anticoagulants.
46
Why should systemic steroids not be prescribed for Degos disease?
Systemic steroids should not be prescribed because they can exacerbate the disease.
47
What is the mechanism of action of eculizumab, and in which phase of Degos disease is it effective?
Eculizumab is a monoclonal antibody that targets C5, preventing the generation of the complement attack complex C5b-9. It is effective in the acute thrombotic phase of Degos disease but has no effect on occlusive fibrointimal arteriopathy.
48
What is the most likely cause of death in a patient with Degos disease presenting with severe intestinal involvement?
The most likely cause of death is severe intestinal involvement leading to complications such as GI hemorrhage, perforation, or peritonitis.
49
What is the significance of parvovirus B19 in the context of Degos disease?
Parvovirus B19 has been associated with acute onset in some cases of Degos disease.
50
What is the significance of parvovirus B19 in the context of Degos disease?
Parvovirus B19 has a known affinity for endothelial cells and may be associated with sudden-onset Degos disease. PCR testing for parvovirus B19 DNA is recommended in such cases.
51
What are the histopathologic features shared between Degos disease, lupus erythematosus (LE), and dermatomyositis (DM)?
Shared histopathologic features include mucin deposition, interface dermatitis, and perivascular lymphocytic infiltrate.
52
What are the major ominous signs or events in Degos disease?
Major ominous signs or events include GI hemorrhage, perforation, peritonitis, and neurologic complications such as hemiparesis, aphasia, and seizures.
53
What is the prognosis for patients with no extracutaneous manifestations within 7 years of diagnosis?
Patients with no extracutaneous manifestations within 7 years have a >90% probability of a benign monosymptomatic cutaneous course.
54
What are the secondary epidermal changes observed in Degos disease histopathology?
Secondary epidermal changes include hyperkeratosis, interface dermatitis, scattered necrotic keratinocytes, and atrophy.
55
What is the clinical significance of mucin deposition in Degos disease histopathology?
Mucin deposition is a characteristic histopathologic finding in Degos disease and is shared with lupus erythematosus and dermatomyositis, suggesting a possible variant relationship.
56
What are the potential neurologic complications of Degos disease?
Neurologic complications include hemiparesis, aphasia, multiple cranial nerve involvement, monoplegia, sensory disturbances, and seizures.
57
What is the role of treprostinil in the treatment of Degos disease?
Treprostinil, a prostacyclin analog, has been effective in some patients with systemic sclerosis/LE overlap and Degos disease-like skin lesions, as well as in a 17-year-old with severe Degos disease progressing on eculizumab.
58
What is the clinical course of extracutaneous manifestations in Degos disease?
Extracutaneous manifestations typically occur within 2-3 years of diagnosis in approximately 30% of cases. Absence of visceral involvement after 2 years indicates a better prognosis.
59
What is the significance of a sudden onset of Degos disease?
A sudden onset of Degos disease may suggest an association with parvovirus B19 viremia, warranting PCR testing for the virus.
60
What is the role of platelet aggregation inhibitors in Degos disease treatment?
Platelet aggregation inhibitors, such as aspirin, clopidogrel, and dipyridamole, are the first-line treatment for Degos disease.
61
What are the diagnostic steps for Degos disease?
Diagnosis is usually established clinically and confirmed with a biopsy. Imaging studies are mandatory if there are signs of GI or CNS involvement.
62
What is the significance of tubuloreticular aggregates in Degos disease?
Tubuloreticular aggregates are occasionally revealed in endothelial cells during electron microscopy, but their significance in Degos disease is uncertain.
63
What is the role of LMWH in Degos disease treatment?
Low molecular weight heparin (LMWH) is worth trying in patients who do not respond to antiplatelet agents.
64
What are the histopathological findings in fully developed lesions of Degos disease?
Key histopathological findings include a cell-poor, wedge-shaped area of mild-to-severe dermal necrosis, occluded vessels with occasional thrombosis, proliferating endothelial cells, and sparse perivascular lymphocytic infiltrate.
65
What are the most frequent complications associated with Degos disease?
GI hemorrhage, perforation, and peritonitis.
66
What is the first-line treatment for Degos disease?
Platelet aggregation inhibitors such as aspirin, clopidogrel, and dipyridamole.
67
What should be monitored in patients with classic Degos disease?
Preventable cardiovascular risk factors, including smoking cessation and lipid levels.
68
What is the role of eculizumab in the treatment of Degos disease?
Eculizumab is a monoclonal antibody treatment that targets C5 to prevent complement attack complex C5b-9 generation.
69
What is the relationship between systemic involvement and prognosis in Degos disease?
Lethality with extracutaneous involvement is greater than 50%, and death typically occurs within 2-3 years due to severe intestinal involvement.
70
What treatments have variable success in managing Degos disease?
Antibiotics, chloroquine, immunosuppressives, phenylbutazone, fibrinolytics, and heparin.
71
What is the significance of the absence of visceral involvement after 2 years in patients diagnosed with Degos disease?
The absence of visceral involvement after 2 years is associated with a better prognosis.
72
What are the first-line treatment options for classic Degos disease?
First-line treatment options include platelet aggregation inhibitors (aspirin, clopidogrel, dipyridamole) and monitoring cardiovascular risk factors.
73
What is the typical progression of lesions in Degos disease?
The typical lesions begin as rose-colored macules that later evolve into porcelain-white atrophic plaques.
74
What is the most common ocular manifestation in Degos disease?
The most common ocular manifestation in Degos disease is retinal involvement.
75
What complication can lead to death among children with Degos disease?
Cerebrovascular events can lead to death among children with Degos disease.
76
What antibodies are seen in some patients with classic Degos disease?
Antibodies seen in some patients include antiphospholipid antibodies and antinuclear antibodies.
77
What is the 5-year survival rate among those with systemic involvement in Degos disease?
The 5-year survival rate among those with systemic involvement is approximately 30-50%.
78
What are the histopathologic findings in Degos disease?
Vascular occlusion, intimal fibrosis, and leukocytoclastic vasculitis.
79
What is the relationship between Degos disease and systemic symptoms?
Skin lesions usually precede the systemic symptoms.
80
What is a common cause of death in Degos disease?
Ischemic or cerebrovascular events.
81
What is the major pathologic mechanism found in Degos disease?
Vascular occlusion.
82
What is the major organ systems involved in Degos disease?
Skin, gastrointestinal, and central nervous system.
83
What is the most common site of cutaneous involvement in Degos disease?
The trunk and extremities.
84
What viral disease should be considered when the onset of symptoms in Degos disease is sudden?
Viral infections such as varicella or herpes zoster.
85
What is the mechanism of action of the treatment that can rescue acutely ill patients in Degos disease?
It acts as an anti-inflammatory and immunosuppressive agent.
86
What are the implications of skin lesions preceding systemic symptoms in Degos disease?
The presence of skin lesions before systemic symptoms suggests a classic form of the disease, indicating that early dermatological manifestations may precede more severe systemic complications.
87
How does the histopathological finding of vascular occlusion relate to the prognosis of Degos disease?
Vascular occlusion is a significant finding that can lead to ischemic complications, correlating with a poorer prognosis.
88
What is the significance of a monocutaneous course lasting at least 7 years in Degos disease?
A monocutaneous course lasting at least 7 years is associated with a better prognosis.
89
What are the major ominous signs in Degos disease that clinicians should monitor?
Clinicians should monitor for signs such as abdominal pain, diarrhea, blood in stool, hemiparesis, paresthesia, and blurred vision.
90
What treatment can effectively rescue an acutely ill patient with Degos disease?
Treatment with systemic glucocorticoids can effectively rescue acutely ill patients by reducing inflammation and immune response.
91
What is the first-line treatment for Degos disease and its dosage?
The first-line treatment typically involves systemic glucocorticoids, with dosages varying based on severity.

Fitzpatrick's Dermatology, 9th Edition (Entrance Exam / Board Exam Prep) (213 decks)

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