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Patient Story

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A 4-year-old boy presented with a one-year history of skin redness and tightening over the right upper extremity. His mother had initially noticed the lesion after applying a temporary tattoo. He complained of occasional itching and pain over the area. His mother was concerned that his arm appeared to be “shrinking.” He had no prior medical problems or preceding infections and his immunizations were up-to-date. He had difficulty grasping a crayon due to involvement of his fingers. On exam, he had a large area of skin tightening extending from the right scapular region and upper arm down to the forearm, index finger and thumb (Figure 178-1). He was referred to a dermatologist who performed a punch biopsy confirming a diagnosis of morphea (localized scleroderma). He was treated with topical fluticasone without benefit and referred to a pediatric rheumatologist for systemic therapy with methotrexate. Serologic testing showed a negative ANA and scleroderma antibody and he has had no features of systemic scleroderma.

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FIGURE 178-1

Morphea on the arm of a 4-year-old boy. He had a large area of skin tightening extending from the right scapular region and upper arm down to the index finger and thumb. (Used with permission from Vidya Raman, MD.)

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Introduction

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Scleroderma (from the Greek scleros, to harden) is a term that describes the presence of thickened, hardened skin. It may affect only limited areas of the skin (morphea), most or all of the skin (scleroderma), or also involve internal organs (systemic sclerosis).

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Epidemiology

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  • Scleroderma is a spectrum of disorders that can be occur at any stage of life, but the clinical patterns seen in childhood differ somewhat from that seen in adults.

  • The prevalence rates of diseases that share scleroderma as a clinical feature are reported ranging from 4 to 253 cases per 1 million individuals of all ages.1

  • The most common form of childhood scleroderma is morphea (localized scleroderma or LSc) which principally involves the skin, subcutaneous fascia, muscle, and bone. It is 10 times more common than systemic sclerosis and may include circumscribed or generalized morphea, bullous morphea, deep morphea, and linear morphea (including the en coup de sabre subtype, characterized by a vertical scar on the forehead resembling a stroke from a sword). 2

  • In a retrospective multicenter review of 750 children with LSc, the most common subtype was linear morphea (65%), followed by circumscribed morphea (26%), mixed subtype (15%), generalized morphea (7%), and deep morphea (2%). Overall, the mean age of presentation was 7.3 years (range 0 to 16 years, median age 6.1 years).3

  • In the US, the incidence of morphea has been estimated at 25 cases per 1 million individuals (of any age) per year.1 Although data are scarce, it is estimated that childhood LSc occurs ...

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