(Kunze-Riehm syndrome)

Very rare. Dermatosis of genetic origin. Possible autosomal dominant transmission.

Presence at birth of  circular and symmetrical skinfolds on the limbs, hands and feet as well as on the trunk, which makes the child looking as a "Michelin Man". They are due to the presence of irregular bundles of smooth muscle fibers or lipomatous structures in the dermis. They tend to decrease during adolescence.

Frequently associated:

-        hypertrichosis

-        facial dysmorphism: bilateral epicanthus, cleft palate, hypoplasia of the lower jaw,  low implanted ears

-        urogenital abnormalities: hypopadias, cryptorchidism

-        heart malformation

-        mental retardation with epilepsy

Anesthetic implications:

preoperative echocardiography; epilepsy, mental retardation, risk of difficult intubation.

References : 

-        Sato M, Ishikawa O, Miyachi Y, Aoki T, Tomomasa T, Nagashima K. 
Michelin tyre syndrome: a congenital disorder of elastic fibre formation ? 
Br J Dermatol 1997 ; 136 : 583-6.

Updated: January 2019