Scleroderma Flashcards Preview

Nelson - Rheumatic Diseases of Childhood > Scleroderma > Flashcards

Flashcards in Scleroderma Deck (18):
1

Juvenile scleroderma encompasses a range of conditions unified by the presence of

Fibrosis of the skin

2

Juvenile scleroderma is divided into 2 major categories

1) Juvenile localized scleroderma (JLS, also known as morphea) 2) Juvenile systemic sclerosis (JSSc)

3

Category of scleroderma which is the predominant type seen in pediatric populations

Localized scleroderma (>95%)

4

Category of scleroderma which is associated with mortality and severe morbidity

Systemic sclerosis

5

MC subtype of localized scleroderma

Linear scleroderma

6

Bluish hue seen around an area of waxy induration

Scleroderma

7

MC extracutaneous manifestation of linear scleroderma

Arthritis

8

MC visceral manifestation of SSc

Pulmonary disease

9

Mortality from JSSc is most commonly a result of

Cardiopulmonary disease

10

Most frequent initial symptom in pediatric systemic sclerosis

Raynaud phenomenon (RP)

11

RP is present in 70% of children when

Months to years before other manifestations

12

Triphasic sequence of blanching, cyanosis, and erythema of the digits induced by cold exposure and/or emotional stress

Raynaud's phenomenon

13

Color changes in RP is brought about by

(1) Initial arterial vasoconstriction, resulting in hypoperfusion and pallor (blanching), (2) venous stasis (cyanosis), and (3) reflex vasodilation caused by the factors released from the ischemic phase (erythema)

14

Color change is classically reproduced by

Immersing the hands in iced water and reversed by warming

15

T/F In RP, the affected area is usually well demarcated and uniformly white

T

16

A condition with episodic color changes and the development of nodules related to severe cold exposure and spasm-induced vessel and tissue damage; this condition has been associated with systemic lupus erythematosus

Chilblains

17

Major criterion (REQUIRED) for JSSc

Proximal skin sclerosis/induration of the skin proximal to metacarpophalangeal or metatarsophalangeal joints

18

JSSc: Most common cause of death is

Heart failure caused by myocardial and pulmonary fibrosis