Cutis marmorata telangiectatica congenita - Clinical features in 35 cases

Objective: To evaluate the distribution of skin lesions, clinical features, and associated abnormalities in children with cutis marmorata telangiectat ica congenita at onset and during follow-up. Design: Retrospective survey of the available medical data with an average follow-up of 1 year 5 months (range, 0-7 years). Setting: Pediatric Dermatology Unit (Department of Dermatovenereology) of t he Sophia Children's Hospital in Rotterdam, the Netherlands. Patients: The diagnosis of cutis marmorata telangiectatica congenita was cl inically established in 35 patients between July 1988 and February 1997. In 33 cases, the typical mottled, blue-violet pattern was present from birth and was readily visible at rest. In 2 cases, the skin lesions initially app eared less reticulated, mimicking a capillary malformation. Results: The skin lesions were almost generalized in 4 children (11%), wher eas they were more localized in the other 31 children (89%). Associated ano malies, usually minor and sometimes questionable, were noted in 80% of the patients. Most patients showed a definite improvement of their mottled vasc ular skin lesions within 2 years. The lesions had totally disappeared, or o nly faded residual lesions remained. Conclusions: We believe that cutis marmorata telangiectatica congenita is a relatively mild condition. The prognosis is usually good, with minor assoc iated anomalies. Improvement of the mottled, vascular pattern is usually ob served within 2 years. We recommend careful clinical examination of all pat ients to exclude any associated anomalies. Patients should be referred to a neurologist or an ophthalmologist only if symptoms are present or if vascu lar lesions are present around the eyes.