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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