Cutis marmorata telangiectatica congenita: report of nine cases and review of the literature.
The major clinical features of nine patients (seven women and two 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 two 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 seven cases, at the age of eight months in one case, and at eighteen months in one case. Three patients had clinical improvement of the skin lesions with age. Eight of the nine 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.
D000328:Adult; D001284:Atrophy; D002196:Capillaries / Q000473:pathology; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D012867:Skin / Q000098:blood supply / Q000473:pathology; D012871:Skin Diseases / Q000151:congenital* / Q000473:pathology; D012880:Skin Pigmentation; D013684:Telangiectasis / Q000151:congenital* / Q000473:pathology