Chapter 16-Derm Dz 6 Flashcards
What are the 3 alternate names for systemic sclerosis?
- progressive systemic sclerosis 2.scleroderma 3.hide-bound disease
Name that disease: dense collagen is deposited in the tissues of the body in extraordinary amounts
systemic sclerosis
What is the prevalence of systemic sclerosis? Gender? Age group?
10-20:1,000,000/yr…female 3-5x, adults
What is often the first sign of systemic sclerosis?
raynaud phenomenon
What is the term for resorption of the terminal phalanges in systemic sclerosis? This along with flexion contractures produce shortened, ______ fingers
acroosteolysis…clawlike
What are the 3 BUZZWORDS for systemic sclerosis’ affect on the face?
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
When the skin changes are confined to the hands,
face, feet, and lower portions of the limbs, the designation of _________ systemic sclerosis is applied.
limited cutaneous
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
diffuse cutaneous
What is the primary cause of death in systemic sclerosis? How many years on avg?
pulmonary fibrosis –> pulmonary HTN and heart failure…within 3 years
What are the 9 oral manifestations of systemic sclerosis? What are the 2 BUZZWORDS?
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
What is the term for a milder condition than systemic sclerosis, affecting only a solitary patch of skin: localized scleroderma aka _______
morphea
What is the buzzword used to describe the clinical appearance of a localized scleroderma / morphea lesion?
“en coup de sabre” (strike of the sword)
What two labs can help dx systemic sclerosis?
Anticentromere antibodies or anti-Scl 70 (topoisomerase I)
Anti-topoisomerase I antibodies (Anti-Scl 70) are
seen more often with ______ cutaneous systemic sclerosis and development of _________
diffuse….pulmonary fibrosis
anticentromere antibodies are usually associated with ______ cutaneous systemic sclerosis (including _____ syndrome) as well as development of _________
limited…(including CREST syndrome)….pulmonary hypertension
What are 2 alternate terms for CREST syndrome?
- Acrosclerosis 2. Limited Scleroderma
CREST is believed to be a variant of what entity?
limited cutaneous systemic sclerosis
What does the acronym CREST stand for?
Calcinosis cutis, Reynaud phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia
Age and gender for CREST?
Female 6th-7th decade
What is the BUZZWORD describing sclerodactyly in CREST syndrome (and systemic sclerosis)?
“claw” deformity
What other entity is on the differential when seeing the telangietasias in CREST syndrome? Which test can help differentiate?
HHT (hereditary hemorrhagic telangiectaisa)…labs for anticentromere antibodies are specific for CREST (and limited cutaneous systemic sclerosis)
Even though the prognosis for CREST is better than systemic sclerosis, which two systemic conditions can still result from CREST?
pulmonary hypertension and primary biliary cirrhosis
What is the acquired dermatologic problem characterized by the development of a velvety, brownish alteration of the skin?
Acanthosis nigricans
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?
malignant acanthosis nigricans…usually GI cancer…20% of cases
What % of people have acanthosis nigricans?
5%
What is the term for acanthosis nigircans that is associated with obesity?
PSEUDOacanthosis nigricans
What are the 4 endocrinopathies associated with acanthosis nigricans? What syndrome? What 2 drug classes?
1.DM 2.addison 3.hypothyroid 4.acromegaly….Crouzon syndrome (fusion of skull bones)….1.oral contraceptives 2.corticosteroids
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?
25-50%…tongue and upper lip..not pigmented