Chap 185 Scleroderma and Raynaud Phenomenon Flashcards
encompasses a range of conditions unified by the presence
of fibrosis of the skin
Juvenile scleroderma
2 major categories of Juvenile scleroderma
juvenile localized scleroderma (JLS , also known as morphea ), which is
largely limited to the skin, and juvenile systemic sclerosis (JSSc ), with
multisystem organ involvement.
predominant type seen
in pediatric populations (>95%),
jls`
___ believed to be a key process in the pathogenesis of both
localized and systemic scleroderma, given the high percentage of affected
children with autoantibodies
Autoimmunity
prevalence of JS
rare, with an estimated prevalence of 1 in 100,000
children.
what is more common in children? LS or SSc
LS
what is the most common subtype
linear scleroderma
initial skin manifestations of localized disease
erythema or a bluish hue seen around an area of waxy
induration; subtle erythema may be the only presenting sign
subtype of LS may have symptoms unique to central nervous system (CNS)
involvement, such as seizures, hemifacial atrophy, ipsilateral uveitis, and
learning/behavioral changes
en coup de sabre
___ causes ulceration of the fingertips
with subsequent loss of tissue pulp and tapered fingers (sclerodactyly )
Severe Raynaud phenomenon
Resorption of the distal tufts of the distal phalanges may occur
(acroosteolysis )
most common visceral manifestation of SSc and
includes both arterial and interstitial involvement
Pulmonary disease
___ is a poor prognostic sign,
developing because of lung disease or independently as part of the vasculopathy
Pulmonary arterial hypertension
Mortality from JSSc is usually a result of ___
cardiopulmonary disease
___is the most frequent initial symptom in pediatric
systemic sclerosis, present in 70% of affected children months to years before
other manifestations
Raynaud phenomenon (RP)
RP refers to the classic triphasic sequence of ___
blanching,
cyanosis, and erythema of the digits induced by cold exposure and/or emotional
stress
true or false: RP is typically independent of an underlying rheumatic disease (Raynaud disease) but can result from rheumatic diseases such as scleroderma, systemic lupus erythematosus (SLE), and mixed connective tissue disease
true
color changes are 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)
Digital ulcers associated with RP are
indicative of ___
underlying rheumatic disease
____ often begins in adolescence and is characterized by
symmetric occurrence, the absence of tissue necrosis and gangrene, and the lack
of manifestations of an underlying rheumatic disease
Raynaud disease
____is a vasospastic
disorder resulting in cool, painless, bluish discoloration in the hands and
sometimes feet despite normal tissue perfusion. It may be exacerbated by
stimulant medications used to treat attention-deficit disorder
Acrocyanosis
____is a
condition with episodic color changes and the development of nodules related to
severe cold exposure and spasm-induced vessel and tissue damage; it has been
associated with SLE
Chilblains
____ comprises a group of
unrelated diseases characterized by patchy or diffuse cutaneous fibrosis without
the other manifestations of scleroderma. These include phenylketonuria,
syndromes of premature aging, and localized idiopathic fibrosis
Pseudoscleroderma
____ is
a transient, self-limited disease of both children and adults that has sudden onset
after a febrile illness (especially streptococcal infections) and is characterized by
patchy sclerodermatous lesions on the neck and shoulders and extending to the
face, trunk, and arms
Scleredema
