CUTIS MARMORATA TELANGIECTATICA CONGENITA - REPORT OF 9 CASES AND REVIEW OF THE LITERATURE

  • 1 January 1984
    • journal article
    • research article
    • Vol. 34  (3) , 305-312
Abstract
The major clinical features of 9 patients (7 women and 2 men) with cutis marmorata telangiectatica congenita (CMTC) were persistent cutis marmorata, spider nevus-like telangiectasia and phlebectasia. Three of the patients had persistent ulcers and atrophy. In 2 patients, atrophie blanche-like lesions developed on the legs with ulcers. No patient had a family history of a similar disorder. Skin lesions were present at birth in 7 cases, at the age of 8 mo. in 1 case, and at 18 mo. in 1 case. Three patients had clinical improvement of the skin lesions with age. Eight of the 9 patients had associated physical anomalies. Biopsy specimens showed an increase in the number and size of the blood vessels in the upper dermis. Alkaline phosphatase staining gave negative results in the dilated blood vessels of the single case in which this histochemical study was done.

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