A 9-month-old girl presented for a well-child check. She was in the 75th percentile for weight and 50th percentile for length. She had excessive skin folds over her arms and legs and bilateral epicanthic folds. She was developmentally appropriate and had no other congenital anomalies. The rest of the physical examination findings were normal.
Her parents reported that the excessive skin folds had been present at birth. There was no similar history in the family.
Circumferential symmetric skin creases are uncommon. They were first described by Ross1 in 1969, when he coined the term “Michelin tire baby syndrome” because of their striking resemblance to the mascot of the French tire manufacturer. The skin creases may be an isolated abnormality or they may be associated with other congenital anomalies. When isolated, the syndrome is inherited as an autosomal dominant trait or it may be sporadic. The skin creases usually resolve over time. The skin creases may be caused by an underlying nevus lipomatosis or smooth muscle hamartoma, but biopsy results are normal in most cases.
Some cases have been associated with a distinct pattern of congenital anomalies, such as cleft palate, abnormal genitals, and distinct facies: short up-slanting palpebral fissures, microphthalmia, epicanthic folds, broad nasal bridge, posteriorly rotated ears, and microstomia. Mental retardation and developmental delay have been reported in a few cases. The clinical course of this subset is not clear as yet and some authors tend to distinguish this as a distinct syndrome: circumferential skin creases Kunze type,2 (named after the first person to report this).
When symmetric skin creases occur in isolation, it is important to reassure the parents of the benign nature of this syndrome and that the creases will resolve over time.
References:
1. Ross CM. Generalized folded skin with an underlying lipomatous nevus. “The Michelin Tire baby.” Arch Dermatol. 1969;100:320-323.
2. Wouters L, Rodriguez Rodriguez CM, Dapena EP, et al. Circumferential skin creases, cleft palate, typical face, intellectual disability and growth delay: “circumferential skin creases Kunze type.” Eur J Med Genet. 2011;54:236-240.
