Scleroderma Flashcards
Juvenile scleroderma encompasses a range of conditions unified by the presence of
Fibrosis of the skin
Juvenile scleroderma is divided into 2 major categories
1) Juvenile localized scleroderma (JLS, also known as morphea) 2) Juvenile systemic sclerosis (JSSc)
Category of scleroderma which is the predominant type seen in pediatric populations
Localized scleroderma (>95%)
Category of scleroderma which is associated with mortality and severe morbidity
Systemic sclerosis
MC subtype of localized scleroderma
Linear scleroderma
Bluish hue seen around an area of waxy induration
Scleroderma
MC extracutaneous manifestation of linear scleroderma
Arthritis
MC visceral manifestation of SSc
Pulmonary disease
Mortality from JSSc is most commonly a result of
Cardiopulmonary disease
Most frequent initial symptom in pediatric systemic sclerosis
Raynaud phenomenon (RP)
RP is present in 70% of children when
Months to years before other manifestations
Triphasic sequence of blanching, cyanosis, and erythema of the digits induced by cold exposure and/or emotional stress
Raynaud’s phenomenon
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)
Color change is classically reproduced by
Immersing the hands in iced water and reversed by warming
T/F In RP, the affected area is usually well demarcated and uniformly white
T
