Epidermal hyperplasia that develops on a limited part of the body: flesh-colored or gray-brownish papulo-warty elevations disposedin linear strips. The hyperplasia is generally present from birth. It seems to be due to mosaism of mutation of various genes.

Very close to the Jadassohn and Solomon syndromes.

It is associated:

-        in 50 % of cases, to neurological anomalies: intellectual deficit, epilepsy, cerebrovascular malformation

-        in 30 % of cases, to ocular anomalies: coloboma of the eyelid, iris and or pupil; dermolipomas of the conjunctiva, enophthalmos, micro - or anophthalmos, cataract

Anesthetic implications:

according to the associated anomalies

References :

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Updated: March 2019