Chapter 16-Derm Dz 6

Question 1

What are the 3 alternate names for systemic sclerosis?

Answer 1

1. progressive systemic sclerosis 2.scleroderma 3.hide-bound disease

Question 2

Name that disease: dense collagen is deposited in the tissues of the body in extraordinary amounts

Answer 2

systemic sclerosis

Question 3

What is the prevalence of systemic sclerosis? Gender? Age group?

Answer 3

10-20:1,000,000/yr…female 3-5x, adults

Question 4

What is often the first sign of systemic sclerosis?

Answer 4

raynaud phenomenon

Question 5

What is the term for resorption of the terminal phalanges in systemic sclerosis? This along with flexion contractures produce shortened, ______ fingers

Answer 5

acroosteolysis…clawlike

Question 6

What are the 3 BUZZWORDS for systemic sclerosis’ affect on the face?

Answer 6

MASK-like facies from deposition on the facial skin

MOUSE facies - Pinched appearance of the nose from atrophy of the ala

“PURSE STRING” appearance at the corners of the mouth

Question 7

When the skin changes are confined to the hands,

face, feet, and lower portions of the limbs, the designation of _________ systemic sclerosis is applied.

Answer 7

limited cutaneous

Question 8

When the skin changes extend past the hands,
face, feet, and lower portions of the limbs and to the trunk and prox limbs the designation of _________ systemic sclerosis is applied

Answer 8

diffuse cutaneous

Question 9

What is the primary cause of death in systemic sclerosis? How many years on avg?

Answer 9

pulmonary fibrosis –> pulmonary HTN and heart failure…within 3 years

Question 10

What are the 9 oral manifestations of systemic sclerosis? What are the 2 BUZZWORDS?

Answer 10

1.microstomia 2.BUZZWORD “purse string” furrows from mouth 3.loss of attached gingiva 4.dysphagia 5.BUZZWORD “boardlike” tongue 6. xerostomia 7.widening of the PDL 8. resorption of the posterior ramus/body (pathologic fracture) 9. tooth resorption

Question 11

What is the term for a milder condition than systemic sclerosis, affecting only a solitary patch of skin: localized scleroderma aka _______

Answer 11

morphea

Question 12

What is the buzzword used to describe the clinical appearance of a localized scleroderma / morphea lesion?

Answer 12

“en coup de sabre” (strike of the sword)

Question 13

What two labs can help dx systemic sclerosis?

Answer 13

Anticentromere antibodies or anti-Scl 70 (topoisomerase I)

Question 14

Anti-topoisomerase I antibodies (Anti-Scl 70) are

seen more often with ______ cutaneous systemic sclerosis and development of _________

Answer 14

diffuse….pulmonary fibrosis

Question 15

anticentromere antibodies are usually associated with ______ cutaneous systemic sclerosis (including _____ syndrome) as well as development of _________

Answer 15

limited…(including CREST syndrome)….pulmonary hypertension

Question 16

What are 2 alternate terms for CREST syndrome?

Answer 16

1. Acrosclerosis 2. Limited Scleroderma

Question 17

CREST is believed to be a variant of what entity?

Answer 17

limited cutaneous systemic sclerosis

Question 18

What does the acronym CREST stand for?

Answer 18

Calcinosis cutis, Reynaud phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia

Question 19

Age and gender for CREST?

Answer 19

Female 6th-7th decade

Question 20

What is the BUZZWORD describing sclerodactyly in CREST syndrome (and systemic sclerosis)?

Answer 20

“claw” deformity

Question 21

What other entity is on the differential when seeing the telangietasias in CREST syndrome? Which test can help differentiate?

Answer 21

HHT (hereditary hemorrhagic telangiectaisa)…labs for anticentromere antibodies are specific for CREST (and limited cutaneous systemic sclerosis)

Question 22

Even though the prognosis for CREST is better than systemic sclerosis, which two systemic conditions can still result from CREST?

Answer 22

pulmonary hypertension and primary biliary cirrhosis

Question 23

What is the acquired dermatologic problem characterized by the development of a velvety, brownish alteration of the skin?

Answer 23

Acanthosis nigricans

Question 24

What is the term for the acquired dermatologic problem characterized by the development of a velvety, brownish alteration of the skin that occurs in conjunction with a malignancy? WHAT TYPE of cancer is most commonly causes this? What % of cases show the cutaneous lesions before the internal malignancy is found?

Answer 24

malignant acanthosis nigricans…usually GI cancer…20% of cases

Question 25

What % of people have acanthosis nigricans?

Answer 25

5%

Question 26

What is the term for acanthosis nigircans that is associated with obesity?

Answer 26

PSEUDOacanthosis nigricans

Question 27

What are the 4 endocrinopathies associated with acanthosis nigricans? What syndrome? What 2 drug classes?

Answer 27

1.DM 2.addison 3.hypothyroid 4.acromegaly….Crouzon syndrome (fusion of skull bones)….1.oral contraceptives 2.corticosteroids

Question 28

What % range of acanthosis nigricans patients have oral lesions? What are the two main sites of oral involvment? Are they pigmented like the cutaneous lesions?

Answer 28

25-50%…tongue and upper lip..not pigmented