Scleroderma

Question 1

characterized by inflammatory, vascular, and sclerotic changes of the skin and various internal organs,especiallythelungs,heart,andGItract.
DIAGNOSIS.

Answer 1

Scleroderma

Question 2

Skin features that are always present.

Answer 2

Skin sclerosis and reynauds phenomenon.

Question 3

Age

Sex

Answer 3

30-50

Female

Question 4

lssc(limited sclerotic scleroderma) Comprises how many percent of pxs.

Answer 4

60%

Question 5

Lesion distribution

Answer 5

long history o Raynaud phenomenon with skin involve- ment limited to hands, feet, face, and fore arms (acrosclerosis) and a high incidence of anticentromeric antibodies

Question 6

Clinical manifestation

Answer 6

Raynaud phenomenon with digital pain, coldness. Pain/stifness of fingers and knees. Migratory polyarthritis. Heartburn, dysphagia, especially with solid oods. Constipation,diarrhea,abdominal bloating,malab- sorption,and weightloss.Exertional dyspnea and dry cough.
SKIN Hands/Feet. Early: Raynaud phenom- enon with triphasic color changes,i.e.,pallor, cyanosis,and rubor

Question 7

what are the 3 triphasic color changes in scleroderma?

Answer 7

pallor, cyanosis,and rubor

Question 8

What do you call the ulcerations in the fingertips?

Answer 8

Rate bite necrosis

Question 9

What do you call the tapering of fingers?

Answer 9

Madonna fingers

Question 10

Facial features.

Answer 10

Early :Periorbitaledema.Late:Edema and fibrosis result in loss of normal facial lines, mask-like (patients look younger than they are),thinning of lips,microsto- mia,radialperioral furrowing and beak-like sharp nose. telangiectasia

Question 11

Difference in distribution of lesions in early and late ssc.

Answer 11

Early: Face, fingers, hands
Late: trunk, upper and lower extremities

Question 12

CREST means.

Answer 12

calcinosis cutis + Raynaud phenomenon + esophageal dysfunction + sclerodactyly + telangiectasia

Question 13

Dermatopath.

Answer 13

Early: Mild cellular
infiltrate around dermal bloodvessels, eccrinecoils, and at the dermal subcutaneous inter- phase.

Late: Broadening and homogenization
o collagen bundles, obliteration and decrease of interbundle spaces,thickening of dermis with replacement of upper or total subcutane- ous atbyhyalinizedcollagen.Paucityo blood vessels, thickening/hyalinization o vessel walls

Question 14

anticentromeric autoantibodies

occur in how many percent in dssc and crest.

Answer 14

Dssc- 21%
CREST - 71%

topoisomerase I (Scl-70) antibodies in 33% of dSSc and 18% o CREST patients.

Question 15

Leading cause or death

Answer 15

Renal dx

Question 16

Treatment

Answer 16

Systemic steroids

10 yr survival rate >50%

Question 17

dssc like condition is due to what exposure?

Answer 17

polyvinyl chloride