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)