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A 35-year-old woman presented with areas of shiny tough skin in patches over her abdomen (Figure 182-1). The patient was otherwise in good health and was puzzled by this new condition. She feared that all her skin would become this way. The skin was slightly uncomfortable but not painful. A 3-mm punch biopsy confirmed the clinical suspicion of morphea or localized scleroderma. The patient was treated with topical clobetasol and calcipotriol with some improvement in skin quality and symptoms. An antinuclear antibody (ANA) test was positive, but she has not developed progressive systemic sclerosis.
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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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- The prevalence rates of diseases that share scleroderma as a clinical feature are reported ranging from 4 to 253 cases per 1 million individuals.1
- Systemic sclerosis has an annual incidence of 1 to 2 per 100,000 individuals in the United States.1 The peak onset is between the ages of 30 and 50 years.1
- In the United States, the incidence of morphea has been estimated at 25 cases per 1 million individuals per year.1
- Worldwide, there are higher rates in the United States and Australia than in Japan or Europe.2
- Pulmonary fibrosis and pulmonary arterial hypertension are the leading causes of death as a consequence of these diseases.3
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- The scleroderma disorders can be subdivided into three groups: localized scleroderma (morphea; Figures 182-1, 182-2, and 182-3), systemic sclerosis (Figures 182-4, 182-5, 182-6, 182-7, 182-8, and 182-9), and other scleroderma-like disorders that are marked by the presence of thickened, sclerotic skin lesions.
- The most common vascular dysfunction associated with scleroderma is Raynaud phenomenon (Figure 182-10). Raynaud phenomenon is produced by arterial constriction in the digits. The characteristic color changes progress from white pallor, to blue (acrocyanosis), to finally red (reperfusion hyperemia). Raynaud phenomenon generally precedes other disease manifestations, sometimes by years. Many patients develop progressive structural changes in their small blood vessels, which permanently impair blood flow, and can result in digital ulceration or infarction. Other forms of vascular injury include pulmonary artery hypertension, renal crisis, and gastric antral vascular ectasia.
- Systemic sclerosis is used to describe a systemic disease characterized by skin induration and thickening accompanied by variable tissue fibrosis and inflammatory infiltration in numerous visceral organs. Systemic sclerosis can be diffuse (DcSSc) or limited to the skin and adjacent tissues (limited cutaneous systemic sclerosis [LcSSc]).
- Patients with LcSSc usually have skin sclerosis restricted to the hands and, to a lesser extent, ...