Crest syndromeM34.1

Variant of systemic scleroderma of the acral (limited) type with combination of:

1. calcinosis metabolica
2. Raynaud's Syndrome
3. "Esophageal Dysfunction"
4. Sclerodactyly
5. Teleangiectasia.

The disease corresponds to the CR(E)ST syndrome with esophageal involvement. (Initial letters list 1-5)

Corresponding to systemic scleroderma, see there. See also Raynaud's phenomenon, telangiectasia, calcinosis cutis.

Casuistically, good successes of cutaneous calcinosis have been described by IVIG therapy over several months (dosage: monthly 4-day cycles with 2 g/day i.v.).

1. Working Group Scleroderma (1986) Clinic of Progressive Systemic Scleroderma. Dermatologist 37: 320-324
2. Chamberlain AJ et al (2003) Successful palliation and significant remission of cutaneous calcinosis in CREST syndrome with carbon dioxide laser. Dermatol Surgery 29: 968-970
3. Genth E, Mierau R, Genetzky P et al (1990) Immunogenetic associations of scleroderma-related antinuclear antibodies. Arthritis Rheum 33: 657-665
4. Lee JB et al (2001) The diagnostic quandary of hereditary haemorrhagic telangiectasia vs. CREST syndrome. Br J Dermatol 145: 646-949
5. Schanz S et al (2008) Response of dystrophic calicification to intravenous immunoglobulin. Arch Dermatol 144: 585-587
6. Schneider GA et al (1998) Seeping gastrointestinal hemorrhage from telangiectasia in CREST syndrome. dermatologist 49: 313-316
7. Thibièrge G, Weissenbach RJ (1910) Une forme de concrétions calcaires sous-cutanées en relation avec la sclérodermie. Bull Soc Mèd (Paris) 30: 10
8. Winterbauer RH (1964) Multiple telangiectasia, Raynaud's phenomenon, sclerodactyly and subcutaneous calcinosis: a syndrome mimicking hereditary hemorrhagic telangiectasia. Bull Johns Hopkins Hosp 114: 361-383