(Epidermal Nevus syndrome)

[MIM 136 200]

Sporadic: it is believed to be caused by an autosomal dominant lethal mutation that survives by somatic mosaicism.  Group of sebaceous nevi, often on the face, arranged parasagittally and linearly following  the lines of Blaschko, often from the hairline to the chin while crossing the orbit; there is frequently an ipsilateral eye anomaly: coloboma of the iris or eyelid, strabismus. Possibly combined with facial asymmetry, epilepsy, mental retardation, or a cardiac malformation (see also:  Schimmelpenning-Feuerstein-Mims syndrome). It evolves in three stages: the first stage is characterized by alopecia with absent or primitive hair follicles and numerous small hypoplastic sebaceous glands. At puberty, the lesions become verrucous with hyperplastic sebaceous glands. Benign or malignant tumors develop later.

Anesthetic implications:

according to the associated anomalies

References :  

• Diaz JH. 
  Perioperative management of infants with the linear naevus sebaceous syndrome of Jadassohn: a report of two cases. 
  Paediatr Anaesth 2000; 10:669-73.

Updated: March 2019