CREST Syndrome PDF Print E-mail
Monday, 08 February 2010 15:48
Those with Limited Coetaneous Scleroderma often have all or some of the symptoms that some doctors call CREST, which can be characterized by the following:

Calcinosis Cutis:

Calcinosis cutis is the term used to describe a group of disorders in which calcium deposits form within connective tissues. It may be focal or widespread, symptomatic or asymptomatic, and when the deposits break through the skin, painful ulcers can result.

Raynaud's Phenomenon:
Raynaud's phenomenon, named after French physician Maurice Raynaud, is characterized by episodic attacks called vasospastic attacks, in which the blood vessels in the digits (fingers and toes) constrict (narrow), usually in response to cold temperatures and/or emotional stress. When this condition occurs on its own, it is called primary Raynaud's phenomenon. When it occurs with another condition such as Scleroderma or Lupus, it is called secondary Raynaud's phenomenon. Occasionally other extremities are also affected.

Esophageal Dysfunction:
Esophageal dysfunction involves the regular contractions occurring in the esophagus or food pipe, which propels the food down towards the stomach. When contractions in the esophagus become irregular, unsynchronized or absent, the patient is said to have esophageal dysfunction. Primary esophageal dysmotility refers to the presence of dysfunction occurring by itself. Secondary esophageal dysfunction refers to esophageal dysfunction occurring as a manifestation of other diseases. In the upper and lower oesophagus, the result can be swallowing difficulties. In the lower oesophagus, the result can be chronic heartburn or inflammation.

Sclerodactyly:
Sclerodactyly is the localized thickening and tightness of the skin of the fingers or toes. It is commonly associated with atrophy of the underlying soft tissues resulting from deposits of excess collagen. The condition makes it harder to bend or straighten the fingers. The skin may also appear shiny and darkened, with hair loss.

Telangiectasia:
Telangiectasia refers to a visibly dilated blood vessels on the skin or mucosal surface, and may be characterized by small red spots appear on the hands and face. Although not painful, these red spots can create cosmetic problems.
People with Limited Coetaneous Scleroderma often have all or some of the symptoms that some doctors call CREST, which can be characterized by the following:

Calcinosis cutis:
Calcinosis cutis is the term used to describe a group of disorders in which calcium deposits form in the skin, through the deposition of hydroxyapatite crystals within cutaneous tissues. It may be focal or widespread, symptomatic or asymptomatic, and when the deposits break through the skin, painful ulcers can result.

An elevated calcium phosphate seen in secondary hypoparathyroidism can lead to nodules of metastatic calcinosis cutis, which tends to be subcutaneous and periarticular. This form is often accompanied by calcification of muscular arteries and subsequent ischemic necrosis. They are four forms of calcinosis cutis: metastatic, dystrophic, idiopathic, and intraepidermal calcified nodule calcinosis.

Raynaud's phenomenon:
Raynaud's phenomenon, named after French physician Maurice Raynaud, is characterized by episodic attacks called vasospastic attacks, in which the blood vessels in the digits (fingers and toes) constrict (narrow), usually in response to cold temperatures and/or emotional stress. When this condition occurs on its own, it is called primary Raynaud's phenomenon. When it occurs with another condition such as Scleroderma or Lupus, it is called secondary Raynaud's phenomenon. Occasionally other extremities are also affected.

Esophageal Dysmotility:
Esophageal dysmotility involves the regular contractions occurring in the esophagus or food pipe, which propels the food down towards the stomach. When contractions in the esophagus become irregular, unsynchronized or absent, the patient is said to have esophageal dysmotility. Primary esophageal dysmotility refers to the presence of dysmotility occurring by itself. Secondary esophageal dysmotility refers to esophageal dysmotility occurring as a manifestation of other diseases.

In the upper and lower oesophagus, the result can be swallowing difficulties. In the lower oesophagus, the result can be chronic heartburn or inflammation.

Sclerodactyly:
Sclerodactyly is the localized thickening and tightness of the skin of the fingers or toes. It is commonly associated with atrophy of the underlying soft tissues resulting from deposits of excess collagen. The condition makes it harder to bend or straighten the fingers. The skin may also appear shiny and darkened, with hair loss.

Telangiectasia:
Telangiectasia refers to a visibly dilated blood vessels on the skin or mucosal surface, and may be characterized by small red spots appear on the hands and face. Although not painful, these red spots can create cosmetic problems.Telagiectasias can be of several types as: Hereditary hemorrhagic telangiectasia, Ataxia - telangiectasia, Spider angioma, Cutis marmorata telangiectatica congenita, Bloom syndrome, Klippel-Trenaunay-Weber syndrome, Sturge-Weber disease, Xeroderma pigmentosa, Nevus flammeus such as port-wine stain.
 
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