Misc II

Question 1

Widespread eruption of small 2-3 mm nonpuritic waxy papules on the face, neck, upper trunk, distal forearms and dorsal surface of hands (sparing the palms) is characteristic of what disease?

Answer 1

Scleromyxedema

Physical exam findings

Question 2

SPEP labs show: over 80% of individuals have IgG monoclonal protein (usually IgG lambda), with 10% developing multiple myeloma.

Answer 2

Scleromyxedema

Question 3

Scleromyxedema skin biopsy shows:

Answer 3

Skin biopsy reveals: normal epidermis, a perivascular mononuclear cell infiltrate, mucin deposition in the papillary dermis, fibroblast proliferation, and fibrosis.

Question 4

Nephrogenic systemic fibrosis causes fibrosis of the skin and internal organs. What causes this?

Answer 4

Exposure to gadolinium contrast while undergoing MRI in a patient with renal insufficiency.

Question 5

Morphea is localized patches of skin thickening, distinguished from scleroderma by:

Answer 5

1) the hands are not involved

2) the patient does not have Raynaud’s phenomenon.

Question 6

Eosinophilic fasciitis is a localized skin thickening, usually on the forearms of young patients. What is associated prior to development of the skin thickening:

Answer 6

strenuous exercise.

Question 7

Treatment for Raynaud’s with digital ulcerations:

Answer 7

calcium channel blockers
nitric oxide paste on the proximal fingers
phosphodiesterase type 5 inhibitor like sildenafil (Viagra)
Pentoxifylline helps red blood cells maneuver more easily
If high risk of losing finger, alprostadil helps vasodilation.

Question 8

Acute elevation in blood pressure with multiple fibrotic layers of blood vessels in the kidneys (onion skinning) is associated with what disease.

Answer 8

Scleroderma renal crisis.

Question 9

Scleroderma renal crisis is associated with what type of anemia:

Answer 9

Microangiopathic hemolytic anemia.

Blood smears will show shistocytes (destroyed red blood cells).

Question 10

Pulmonary hypertension in limited scleroderma is associated with what antibody?

Answer 10

anti-centromere positivity.

Question 11

Every patient with scleroderma, no matter what their antibody, should be monitored with

Answer 11

Chest xray
Echocardiogram increased right ventricular systolic pressure and pulmonary hypertension
Pulmonary function tests