Crest Syndrome

CREST is a form of Systemic Sclerosis which is characterized by:

Calcinosis- (calcium deposits) usually in the fingers
Raynaud's phenomenon
Oesophageal dysmotility-loss of muscle control of the Esophagus which can cause difficulty swallowing Sclerodactyly -a tapering deformity of the bones of the fingers and
Telangiectasia-small red spots on the skin of the fingers, face, or inside of the mouth

Calcinosis cutis: Calcinosis cutis is a term used to describe a group of disorders in which calcium deposits form in the skin. Virchow initially described calcinosis cutis in 1855. It is the deposition of hydroxyapatite crystals within cutaneous tissues. It may be focal or widespread, symptomatic or asymptomatic. 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. The forms vary as according to their cause of symptoms.

Raynaud's phenomenon: Raynaud's phenomenon (RAY-noz) named after French physician Maurice Raynaud (1834 - 188) which 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 (usually idiopathic). When it occurs with another condition such as scleroderma or lupus, it is called secondary Raynaud's phenomenon. Occasionally other extremities are also affected. It appears to be more common in people who live in colder climates.

Esophageal Dysmotility: Esophageal motility involves the regular contractions occurring in the esophagus or food pipe, which propels the food bolus forward 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.

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.

Telangiectasia: A telangiectasia refers to a visibly dilated blood vessel on the skin or mucosal surface. Telagiectasias can be of several types as: Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome), Ataxia - telangiectasia, Spider angioma, Cutis marmorata telangiectatica congenita (an inherited disorder), Bloom syndrome, Klippel-Trenaunay-Weber syndrome, Sturge-Weber disease, Xeroderma pigmentosa, Nevus flammeus such as port-wine stain.