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ophthalmologic consultation should be obtained to exclude other complications such as
retinal vasculitis or retinal vascular occlusion. If other causes are excluded, a child with
a severe headache may be treated for a suspected acute migraine with analgesics and
antiemetics (see Chapters 59 Pain: Headache and 97 Neurologic Emergencies ).

OTHER SYSTEMIC CONNECTIVE TISSUE DISEASES
Scleroderma and Mixed Connective Tissue Disease
Goals of Treatment
The goals of treatment are to control symptoms and allow the patient to maintain
function while simultaneously monitoring for the development of complications.
CLINICAL PEARLS AND PITFALLS
Complications related to juvenile systemic sclerosis (JSSc) should be
considered if a child with localized scleroderma presents with acute clinical
decompensation.
Mixed connective tissue disease is a systemic autoimmune process similar
to SLE and characterized by high-titer anti-RNP antibodies and often
complicated by interstitial lung disease.

Scleroderma
Scleroderma, or hardening of the skin, is most commonly a process restricted to the
skin and subcutaneous tissues in children. Various conditions are included within the
category of scleroderma, as listed in Table 101.4 . Localized scleroderma (LSc) is the
more prominent form found in childhood. The lesions may be one of five types.
Circumscribed morphea is a focal ivory-white patch with a violaceous or erythematous
rim; it is often a single lesion on the trunk, although generalized morphea also occurs in
children. Pansclerotic morphea is circumferential involvement of the limb(s) affecting
the skin, subcutaneous tissue, muscle, and bone. Linear scleroderma causes scarring,
fibrosis, and atrophy that crosses dermatomes. Involved skin develops a “hidebound”
appearance due to tethering of the subcutaneous tissues to deeper structures. It may
extend to involve an entire extremity ( Fig. 101.3 ) and to affect underlying muscle and
bone, leading to flexion contractures, leg-length discrepancies, and atrophy of an

extremity. A variant affecting the forehead is called scleroderma en coup de sabre ; this
form may involve underlying skull and nervous tissue, as well as the skin. Finally, there
is a mixed type which is a combination of two or more of the previous subtypes.
Although localized forms of scleroderma are generally not associated with internal
organ involvement, one large pediatric cohort found nearly a quarter of patients to have