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Morphea is a chronic autoimmune disease characterized by sclerosis of the skin. The term “localized scleroderma” is also used in an attempt to highlight the systemic features of morphea. This causes confusion with systemic sclerosis (scleroderma) often resulting in unnecessary evaluation and anxiety. It is the opinion of the authors this term should be avoided. Morphea itself has a spectrum of manifestations ranging from skin only to multiple organ involvement. Of note, organ involvement in morphea is distinctly different from systemic sclerosis (Box 64-1).
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Morphea has an estimated incidence of 2.7 per 100,000 with a female:male ratio of 2 to 3:1.1 Morphea is more common in Caucasians.2–5 The relative frequency of the different subtypes varies between studies. This is likely due to use of different classification systems. Twenty to thirty percent of morphea begins in childhood, but it can occur at any age.2–5 Linear morphea is the most common pediatric subtype (although all subtypes occur at any age).1,2,6 Twenty-five to eighty-seven percent of pediatric cases are linear morphea, with limb or trunk involvement in approximately 70%–80% and en coup de sabre (ECDS) or progressive facial hemiatrophy (PHA; formerly described as Parry–Rhomberg) in 22%–30%.1,2,4,5,7,8 In adults, circumscribed and generalized subtypes predominate. Deep morphea/morphea profunda is uncommon in both adults and children with a frequency of 2%–4%.1,2,4,7,8
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Periods of disease activity vary from 3 to 6 years, but reactivation after periods of remission occurs in 20%.2,8 (eFig. 64-0.1). Others have a chronic course persisting ...