Scleroderma

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Types of Scleroderma

Scleroderma is chronic and is seen in two forms: systemic or localized. Systemic scleroderma can be further categorized as either diffuse scleroderma or CREST syndrome.

Systemic scleroderma
Diffuse scleroderma is seen in 10% of systemic scleroderma, often progressing quickly, and is potentially fatal. It can affect large areas of skin, causing thickening and hardening of the skin (sclerosis), abnormal changes with the arteries, joint problems, and dysfunction of major organs, such as the intestinal tract, heart, lungs, and kidneys.

CREST syndrome is sometimes referred to as limited cutaneous scleroderma and typically affects specific areas of the body such as the skin of the fingers, hands, lower arms and legs, elbow joints, and face. It progresses gradually. CREST syndrome, seen in 90% of cases of systemic scleroderma, is defined as follows:

  • Calcinosis: Small calcium deposits that develop under the skin, primarily on the fingers, knees and elbows. They can become infected and can cause painful, open sores when they break through the skin.
  • Raynaud phenomenon: Blood vessels in the hands and feet contract in response to cold or stress with this condition. When this happens, blood flow decreases and the affected fingers and toes turn white, then blue. When blood flow returns to normal, they turn red. The recurrent or prolonged interruption in blood flow can damage tissues, leading to sores and, in some severe cases, to gangrene and the need for amputation. Mild to moderate Raynaud phenomenon is relatively common, and only a small percentage of those who have it also have scleroderma. On the other hand, up to 90% of those with limited cutaneous scleroderma will have Raynaud phenomenon.
  • Esophageal dysfunction: Many of those with scleroderma have difficulty swallowing due to muscle dysfunction in their esophagus and may have acid reflux and chronic heartburn.
  • Sclerodactyly: This refers to the development of tight, thick skin causing deformity of the fingers that are in a fixed, semiflexed position. There may also be loss of tissue on the finger pads (digital pitting) and shrinkage giving them a sausage-like appearance with fingernails that may curve about the fingertip. The skin may be shiny and discolored.
  • Telangiectasia: Associated with tiny red spots on the face and hands caused by the swelling of small blood vessels.

In more severe cases of systemic scleroderma, organ involvement may include:

  • Lungs: Fibrosis can stiffen lung tissue, making it harder to breathe and leading to low oxygen levels, causing fatigue and shortness of breath, and to pulmonary hypertension (high blood pressure in the lungs), which can cause congestive heart failure. Lung dysfunction is the leading cause of death in systemic scleroderma.
  • Kidney: Scleroderma can cause acute renal crisis that can be life-threatening. It can also cause hypertension and chronic renal dysfunction.
  • Heart: Less common, and more likely to occur after several years, heart involvement can lead to heart palpitations and congestive heart failure.

Complications associated with systemic scleroderma that may also be associated with other disorders and present with overlapping clinical features include:

  • Dental problems: Scleroderma of the face may make the mouth opening narrower, making teeth cleaning and dental care more difficult.
  • Mask-like face: Tightening of the skin may cause an expressionless, mask-like face.
  • Carpal tunnel syndrome
  • Erectile dysfunction, sexual dysfunction in women
  • Hypothyroidism
  • Hearing loss
  • Depression

Localized scleroderma
Localized scleroderma, also known as morphea, is characterized by the deposition of excessive collagen in the skin, causing one or many patches or lines of thickened, hardened skin. The skin may be reddened or white with purplish borders. In some cases, large areas of the skin may have patterns of hypo- and hyper-pigmentation. Morphea lacks some of the characteristics seen in the systemic form, such as sclerodactyly, Raynaud phenomenon, fingernail changes, telangiectasia, and organ involvement.

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