Cutis marmorata telangiectatica congenita: clinical findings in 85 patients

Cutis marmorata telangiectatica congenita (CMTC) is an uncommon, sporadic, congenital cutaneous condition presenting with persistent cutis marmorata, telangiectasia, phlebectasia and possible ulceration of the involved skin, skin atrophy, and undergrowth of the involved extremity. To further the current understanding of this disorder, we analyzed the clinical features of a large series of patients with CMTC. The files of 85 patients with CMTC who were examined in our center over the last 20 years were reviewed. The data recorded included patient sex, age at onset, nature and distribution of the lesions, associated abnormalities, and course of disease. CMTC was characterized by early recognition of the lesions (94% at birth) and equal sex distribution. The lesion was unilateral in 65% of patients and most commonly involved the limbs (69%). Additional capillary lesions, noted in 20% of patients, may well have been an extension of the CMTC itself. Associated anomalies were found in 18.8% of patients. Almost half of the patients showed a definite improvement in the reticular vascular pattern on follow-up. No familial cases of CMTC were noted. CMTC is more prevalent than previously recognized, affects both sexes equally, and has a mostly localized distribution; additional vascular lesions are associated with the disorder, but other anomalies occur less often. Prognosis is generally good.