Syn .: CREST syndrome, Th ibierge-Weissenbach syndrome, Winterbauer syndrome.
Def .: It is a type of limited scleroderma with minimal induration and severe vascular changes. The syndrome name is an acronym of the first letters of the main symptoms of the syndrome, such as calcinosis, Raynaud’s phenomenon (Raynaud’s phenomenon), esophageal disorders (esophagus), sclerodactyly (sclerodactyly) and telangiectasia (teleangiectasia).
Epid .: No specific data available. This syndrome, like lSSc, is much more common in women. CREST syndrome is rare in children.
Etiol .: Systemic scleroderma.
Localization: Multiple sclerosis affects the distal parts of the hands, usually
do not exceed the border of the metacarpophalangeal joints.
Clinical: Hard swelling and vascular changes (very intense Raynaud’s phenomenon) are found in the fingers, resulting in poorly healing erosions or ulcerations on the fingertips. In a few cases, necrosis of individual phalanges occurs. There are telangiectasias on the backs of the hands and on the palms. The face may be masked or unchanged except for numerous telangiectasias. There are radial furrows around the mouth. In most cases, calcium deposits are present, especially around the joints (elbows, knees) in the soft tissues, which can be very abundant. The piercing of calcium masses is accompanied by great pain and difficult to heal ulcers.
Hist .: Same picture as for systemic scleroderma.
DL: Tests as in systemic scleroderma. Attention should be paid to the diagnosis of primary pulmonary hypertension and arrhythmias. In some cases of CREST syndrome (10-15%), chronic biliary cirrhosis (PBC) and Sjögren’s syndrome coexist.
DI: Anti-centromere antibodies are an immunological marker and are found in 50-96% of cases (met IIF). Mitochondrial antibodies are also found in patients with coexisting PBC.
DR: Differentiation is related to Raynaud’s disease, in which there is no hardening of the skin and no ACA antibodies.
Healing: Mainly vascular drugs: pentoxifylline, prostacyclin and vit. E and Piascledina. It should be remembered that the prostacyclin infusion is temporary.
Year: It is the mildest form of systemic scleroderma. In cases of primary pulmonary hypertension or primary biliary cirrhosis, the prognosis is more severe.
DIG. Z-38. The CREST team. The swelling hardened the fingers.
DIG. Z-39. The CREST team. Calcium deposits.
Lit.: [1] Jabłońska S. (red.): Scleroderma and pseudoscleroderma. Dowden, Hutchinson and Ross, Stroudsburg PA 1975. [2] Clements Ph.J., Furst D.E.: Systemic sclerosis. Wyd. 2, Lippincott, Williams & Wilkins, Philadelphia 2004.
Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House