Schimmelpenning-Feuerstein-Mims syndrome

[MIM 163 200, 156 630]

(epidermal nevus syndrome, Solomon syndrome, Solomon-Fretsin syndrome)

See also Jadassohn Nevus which is very close (less severe form ?)

Rare. Neurocutaneous syndrome. Sporadic. Caused by somatic mutations (mosaic) of the HRAS gene (11p15.5), the KRAS gene (12p12.1) or the NRAS gene (1p13.2).

Clinical features:

-        in children: yellow-orange to pink plate

-        in adolescents: following hormonal stimulation of the sebaceous glands at puberty, appearance of epidermic papillomatous hyperplasia which covers the nevus

-        later, in rare cases: benign or malignant tumours (basocellular epithelioma) at this level

A sebaceous nevus is present in 1 to 3 infants on 1000: it is estimated that about 10 % of them develop extra-cutaneous signs.

Anesthetic implications:

ECG and echocardiography; epilepsy

References :  

Updated: July 2018