Incontinentia pigmenti is a rare genodermatosis also called Bloch-Sulzberger syndrome or Bloch-Siemens, that shows early at birth or in the neonatal period.In its classical form,the cutaneous symptomatology develops through three steps. At third-sixth month of life dark pigmented spots appear at the upper body level, positioned like a vortex, a whirl or spurts. Incontinentia pigmenti is linked, in 80% of occurrencies, to other anomalies: - Neurological anomalies in 30% of occurrencies, with motor-spastic involvment, convulsions, intellective impairment. - Ocular anomalies in 35% of cases, with 7,5 of induced blindness ( cataract, retinitis, uveitis, optic nerve atrophy ). - Dental anomalies in 65% occurrencies. - Alopecia 38% of cases. - Onychodystrophy 7% of occurrencies. Late studies in molecular genetics identified the origin of incontinentia pigmenti in the failure of a gene named NEMO, placed on X-chromosome.

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