Incontinentia Pigmenti

**Definition:** A genodermatosis occurring mostly in females and characterized by skin changes in three phases - vesiculobullous, verrucous papillomatous, and macular melanodermic. Hyperpigmentation is bizarre and irregular. Sixty percent of patients have abnormalities of eyes, teeth, central nervous system, and skin appendages.

**Tree numbers:** - C16.131.077.445 - C16.131.831.580 - C16.320.850.420 - C17.800.621.497 - C17.800.804.580 - C17.800.827.420

**Synonyms:** - Bloch-Sulzberger Syndrome - Bloch-Siemens Syndrome - Bloch-Siemens-Sulzberger Syndrome