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MORPHEA

AT-A-GLANCE

  • Occurs in children and adults.

  • Linear subtype predominates in children.

  • Circumscribed and generalized predominate in adults.

  • A self-limited or chronically relapsing autoimmune disorder targeting the skin with the following major features:

    • Inflammatory, sclerotic, atrophic phases.

    • Thickened sclerotic skin.

    • Systemic disease, including arthritis and neurologic disorders, in a minority.

    • Differentiated from scleroderma by lack of acrosclerosis/sclerodactyly.

  • Complications may cause significant irreversible cosmetic and functional impairment including the following:

    • Atrophy of dermis, fat, and subcutaneous structures.

    • Contracture.

    • Limb-length discrepancy.

    • Bony abnormalities.

  • Effective treatment reduces signs of disease activity and does not produce complete resolution of lesions. Treatment based on the following:

    • Disease subtype.

    • Depth of involvement.

    • Stage (inflammatory, sclerotic, atrophic).

    • Potential for complications.

Morphea is a chronic inflammatory disease characterized by sclerosis of the skin. The term localized scleroderma is also used in some texts. This causes confusion with systemic sclerosis (scleroderma), often resulting in unnecessary evaluation and anxiety. It is the opinion of the authors that the term localized scleroderma 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 (Table 64-1).

Table 64-1Differential Diagnosis of Morphea

EPIDEMIOLOGY

Morphea has an estimated incidence of 2.7 per 100,000 with a female-to-male ratio of 2 to 3:1.1 Morphea is more common in whites, although population-based studies have not verified this observation.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 percent to 87% of pediatric cases are linear morphea, with limb or trunk involvement in approximately 70% to 80% and en coup de sabre or progressive hemifacial atrophy (formerly described as Parry-Romberg syndrome) in 22% to 30%.1,2,4,5,7,8 In adults, circumscribed and generalized subtypes predominate.5 Deep morphea/morphea profunda is uncommon in both adults and children, with a frequency of 2% to 4%.1,2...

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