Systemic abnormalities associated with cutis marmorata telangiectatica congenita.

Nearly 68% of patients with cutis marmorata have a congenital abnormality, the most common being body asymmetry. Cutis marmorata has also been shown to be associated with the chiari malformations, macrocephaly, hemimegalencephaly, bilateral cortical dysplasia, calvarial haemangioma and cavum septi pellucidum cysts.2 In fact, this subset of patients may have developmental delay and are now classified under a separate subset: macrocephalycutis marmorata telangiectatica congenita (MCMTC). Similarly, ocular abnormalities such as lateonset paediatric glaucoma have been reported in some patients.3 Cardiac defects such as atrial septal defects and pulmonary vein stenosis may also occur. Haematologic abnormalities such as myelodysplasia and even gastro-intestinal abnormalities such as imperforate anus may occur.4 Hypospadias is another common abnormality seen in association with cutis marmorata. Overall, the prognosis in cutis marmorata is very good, as almost all patients show an improvement in the cutaneous lesions during infancy. Rarely, serious complications such as bleeding episodes, gangrenous ulceration and hypovolaemic shock may occur.5 The disease may occasionally be fatal, especially in patients with M-CMTC.2