Cutis marmorata telangiectatica congenita (CMTC) is a rare capillary malformation characterised by persistent reticulated marbled erythema. It tends to be associated with cutaneous atrophy, ulcerations and body asymmetry. CMTC is usually reported to be a benign condition; however, associated anomalies are not rare. Here, we have compiled information on published CMTC patients with the aim to evaluate the proposed diagnostic criteria by Kienast et al. and address the clinical manifestations, associated anomalies, differential diagnoses, management and prognosis. Our review is based on a search of the PubMed database which retrieved studies between 1922 and April 2019. The search yielded 148 original articles with a total of 485 patients.
Of the identified patients, 24.5% had generalised CMTC, 66.8% had localised and 8.7% had a non-specified distribution of CMTC. Associated anomalies were observed in 42.5% of patients, predominantly body asymmetry and neurological defects like seizure and developmental delay. Fewer patients (10.1%) had ophthalmological defects, usually glaucoma. The major criterium \"absence of venectasia\" was not met in 20.4% of patients.
We suggest that children with CMTC should be referred to an ophthalmologist for regular follow-up, and children with CMTC affecting the legs should be monitored for leg length discrepancy throughout the growth period. Furthermore, we suggest reconsideration of the major criterium \"absence of venectasia\" from the proposed diagnostic criteria, and instead include body asymmetry.

BACKGROUND: Cutis marmorata telangiectatic congenital or Van Lohuizen syndrome is a rare vascular disorder that may be associated with other congenital malformations. Around 300 cases have been reported so far.
METHODS: We present a 4-year-old girl with Van Lohuizen syndrome of the leg, but without any other malformations.
CONCLUSIONS: Neonatal lupus erythematosus may resemble congenital vasculopathy, but histopathology and immune-serology are characteristic.

BACKGROUND: Cutis marmorata telangiectatica congenita (CMTC) is a sporadic congenital skin vascular abnormality. Significant number of patients has other congenital anomalies.
METHODS: We report a case of a preterm male newborn with cutis marmorata pattern presented on the skin of the face, right side of front of the trunk, whole back, glutei and both legs. Besides, microretrognatia and asymmetric, bad-formed, low-set ears were notable. Ophthalmologic findings showed visual impairment and pale optic nerve papilla. Monitoring of child showed mental underdevelopment and motor retardation.
CONCLUSIONS: CMTC is a rare finding with good prognosis of skin malformations, with an obligation of dermatologist and paediatrician to investigate other associated congenital anomalies.