22 - 146 - MALIGNANT ATROPHIC PAPULOSIS (DEGOS DISEASE)

Question 1

True of degos disease except

a. Majority of cases are autosomal dominant pattern of inheritance.

b. A combination of prothrombotic factors possibly plays a role in triggering the fullblown disease.

c. Men are more affected than women.

d. Benign forms without extracutaneous involvement are possible.

Answer 1

A

Page 2630. The majority of cases are sporadic, but familial cases have been described, and most of these cases are consistent with an autosomal dominant pattern of inheritance.

Question 2

The most common ocular manifestation of Degos disease is:

a. Avascular patch on the conjunctivae

b. Posterior uveitis

c. Inflammation of the optic nerve

d. All of the above.

Answer 2

A

Page 2631. The most common manifestation is an avascular patch on the conjunctivae, but sclerae, episclera, retina, choroids, and optic nerves may be affected.

Question 3

Which is not found in the histology of Degos Disease?

a. Cell-poor, wedge-shaped area of mild-to-severe dermal necrosis

b. Dermal edema and copious mucin deposition, extending from the papillary dermis to the deep reticular dermis

c. Occluded vessels with occasional thrombosis, thickened vessel walls with intimal fibrosis

d. Full-blown leukocytoclastic, neutrophilic vasculitis

Answer 3

D

Page 2632. Full-blown leukocytoclastic, neutrophilic vasculitis is never found in patients with malignant atrophic papulosis, signifying this entity should not be classified as vasculitis.

Question 4

True of Degos Disease except

a. If no extracutaneous manifestations have occurred 7 years after diagnosis, it is probably benign

b. Lethality is greater than 50% if patients present with extracutaneous involvement

c. Familial disease bears a poorer prognosis

d. GI hemorrhage, perforation, and peritonitis are the most frequent complications of the disease and are the major ominous events

Answer 4

C

Page 2633. Familial Degos disease bears a better prognosis.

Question 5

First-line treatment of patients with Degos disease

a. Platelet aggregation inhibitors

b. Warfarin

c. Anti-malarials (chloroquine)

d. Heparin

Answer 5

A

Page 2634. First-line treatment of patients with Degos disease should include platelet aggregation inhibitors (aspirin, clopidogrel, dipyridamole)

Question 6

Malignant atrophic papulosis (Degos disease) is a rare, primary vasoocclusive disorder, affecting mainly what organ systems?

Answer 6

skin, the GI tract, and the CNS

• cutaneous lesions varies from a few to more than 100.
• Cutaneous findings usually precede the systemic manifestations.
• GI findings include **bowel perforation and peritonitis **
• CNS findings include hemorrhagic or ischemic stroke
• Cutaneous lesions rarely occur simultaneously or after GI or CNS involvement

Question 7

the clinically distinctive lesion of so-called Degos disease is a marker of what?

Answer 7

cutaneous thromboobliterative vasculopathy

Question 8

Question 9

characeristic lesions of Malignant atrophic papulosis (Degos disease)

Answer 9

porcelain-white, atrophic papules with a rim of rosy erythema and/or telangiectasias

• Cutaneous lesions start as crops of 2 to 10 mm, largely asymptomatic or mildly pruritic, rose-colored macules that progress quickly to round, smooth, often dome-shaped, firm papules (Fig. 146-2).
• Some lesions display central umbilication and/or necrosis (Fig. 146-3).
• These lesions evolve over days or weeks to porcelain-white, atrophic papules with a rim of rosy erythema and/or telangiectasias (Fig. 146-4).
• A fully developed lesion closely resembles the changes of atrophie blanche.
• In time, the reddish border disappears, and only a varicelliform white scar remains.
• The lesions of Degos disease are typically discreet, but they may coalesce, leading to polycyclic atrophic areas or ulcerations.
• The lesions are most often located on the trunk and limbs.
• Palms, soles, face, scalp, and genitalia are usually spared

Question 9

. Exclusive localization on what part is suggestive of connective tissue disease rather than Degos disease?

Answer 9

acral location

Question 10

most common ocular manifestation of Degos disease

Answer 10

avascular patch on the conjunctivae

• sclerae, episclera, retina, choroids, and optic nerves may be affected

Question 11

histopath findings of Degos disease

Answer 11

• Fully developed lesions of Degos disease demonstrate a **cell-poor, wedge-shaped area of mild-to-severe dermal necrosis with dermal edema **and copious mucin deposition, extending from the papillary dermis to the deep reticular dermis
• At the base of the lesion, occluded vessels with occasional thrombosis, thickened vessel walls with intimal fibrosis, proliferating endothelial cells, and a sparse perivascular lymphocytic infiltrate can be found
• A full-blown l**eukocytoclastic, neutrophilic vasculitis is never found **in patients with malignant atrophic papulosis

Question 12

Question 13

most frequent complications of Degos Disease

Answer 13

GI hemorrhage, perforation, and peritonitis

• major ominous events

Question 14

the major complication of Degos disease

Answer 14

death related to an ischemic cerebral or GI event

Question 15

First-line treatment of patients with Degos disease should include what medications?

Answer 15

platelet aggregation inhibitors (aspirin, clopidogrel, dipyridamole)

Question 16

targets C5 and inhibits its cleavage to C5a and C5b by the C5 convertase, preventing the generation of the terminal complement complex C5b-9

Answer 16

Eculizumab

Question 17