A 8-month-old female baby presented with cushingoid facies,
with large skin folds involving the extremities and trunk
since birth (Fig. 1). The child was born to healthy
parents in a non-consanguineous marriage. The child weighed
8 kgs, was exclusively breast fed and developmentally normal
and had no dysmorhic features. There was no history of
similar complaints in other family members. Serum cortisol
levels were normal. No abnormality was detected on
abdominopelvic sonography.Skin biopsy revealed normal
histology.
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Fig. 1 Michelin tyre baby
with skin folds over trunk.
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Michelin tyre baby syndrome is a rare
benign genodermatosis resulting in multiple, symmetric skin
folds since birth. This was first described by Ross in 1969,
deriving its name from resemblance of the skin folds to the
mascot of a French tire company. The skin folds are
predominantly present on the extremities and trunk rarely on
the palms and soles. Multiple congenital anomalies have been
associated including low set ears, congenital heart defects,
cleft palate and undescended testis. The syndrome may be
familial. Skin biopsy may be normal. However there are
biopsy reports suggesting diffuse lipomatous nevus in
underlying dermis or smooth muscle hamartoma. Scarring
instead of increased adipocytes have also been reported. The
condition is benign and the skin folds gradually disappear.
Differential diagnosis includes congenital amniotic bands;
however these are usually solitary and limited to limbs.
Beare Stevensons cutis gyrate syndrome is another syndrome
characterized by dermatomegaly which is limited to scalp,
forehead, face and neck.