[Cutis marmorata telangiectatica congenita. Review of 33 cases].

INTRODUCTION: Cutis marmorata telangiectatica congenita (CMTC) is an uncommon congenital vascular malformation characterized by the presence of a localized or generalized pattern, frequently asymmetrical, with reticulated, erythematous or violaceous macules, present at birth or shortly thereafter.

PATIENTS AND METHOD: Retrospective study of 33 cases of CMTC diagnosed between 1994 and 2007 in our hospital. Clinical and follow-up data were recorded in all cases. In some patients additional tests were performed.

RESULTS: Most of the patients (51.5%) were female. In 87.9% of the cases lesions were observed at birth. All the cases were sporadic. CMTC was localized in 72.7% of the patients, being distributed mainly over the lower limbs. Associated anomalies were noted in 60.6%, with predominance of skin alterations (14 cases). Extracutaneous anomalies were present in 11 patients, most commonly atrophy and hypertrophy in the involved limbs. In none of the cases were there ocular or neurological manifestations. The average follow-up time was 14 months. Improvement of the lesions was observed in 45%, and in one case there was complete resolution.

CONCLUSIONS: CMTC is a malformative disease which is frequently associated with other abnormalities. An appropriate assessment and follow-up of these patients is advisable. Performing a thorough medical history, including full physical examination is necessary. If the head is affected, ocular and neurological examination should be performed. The prognosis is generally good, with a tendency to improvement or disappearance in most cases.