Juvenile scleroderma syndromes are multisystem autoimmune rheumatic
diseases whose unifying characteristic is the development of hard skin
before age 16. They can be separated into two main categories: those
with diffuse skin sclerosis involving many sites of the body together
with internal organ involvement (juvenile systemic sclerosis [JSSc])
and those with circumscribed skin induration but no vascular or internal
organ involvement (juvenile localized scleroderma [JLS]).
Juvenile systemic sclerosis (JSSc) is a chronic multisystem connective
tissue disease characterized by the symmetrical thickening and hardening
of the skin, associated with fibrous changes in such internal organs
as the esophagus, intestinal tract, heart, lungs and kidneys, plus
arthritis and myositis. A Committee on Classification Criteria for JSSc,
including pediatricians, rheumatologists, and dermatologists, recently
proposed new classification criteria (Table 206-1).1
Table 206-1. Preliminary
Classification Criteria for Juvenile Systemic Sclerosis |Favorite Table|Download (.pdf)
Table 206-1. Preliminary
Classification Criteria for Juvenile Systemic Sclerosis
of the skin|
|Vascular||Raynaud phenomenon |
|Nail fold capillary abnormalities|
|Digital tip ulcers|
|Gastroesophageal reflux |
|New onset arterial hypertension|
|Respiratory||Pulmonary fibrosis (HRCT/X-ray)|
|Reduced diffusion capacity for carbon monoxide (DLCO)|
|Musculoskeletal||Tendon friction rubs|
|Carpal tunnel syndrome|
|Serology||Antinuclear antibodies. |
|SSc selective autoantibodies (anticentromere, antitopoisomerase
I, antifibrillarin, anti-PM-Scl, antifibrillin, or antiRNA polymerase
I or III)|
Systemic sclerosis is a rare condition in any age group, with
an estimated annual incidence ranging from 0.45 to 1.9 in 100,000,
and a prevalence of approximately 15 to 24 in 100,000.2
Onset in childhood is particularly uncommon: children under age
16 account for less than 5% of all cases,3 and
fewer than 10% develop systemic sclerosis before age 20.4,5 Juvenile
systemic sclerosis develops at a mean age of 8.1 years, with a peak
incidence between ages 10 and 16.3 The disease
is almost 4-fold more prevalent in females, but there is no recognized
Children developing scleroderma typically present with Raynaud
phenomenon and skin changes (eFig. 206.1).
Raynaud phenomenon is the first sign of the disease in 70% of
patients and in 10% it is complicated by digital infarcts (eFig. 206.2). It is more common in the fingers
but can be observed in other acral regions including the toes, ears,
lips, tongue, and tip of the nose.
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