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Concept information

Preferred term

Incontinentia Pigmenti  

Type

  • Topical Descriptor

Entry terms

  • Bloch-Siemens-Sulzberger Syndrome
  • Bloch-Siemens Syndrome
  • Bloch Sulzberger Syndrome
  • Bloch-Sulzberger Syndrome
  • Syndrome, Bloch-Sulzberger

Note

  • multiple skin & other abnorm; do not confuse with INCONTINENTIA PIGMENTI ACHROMIANS see PIGMENTATION DISORDERS

Scope note

  • 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.

History note

  • 91(87); was see under PIGMENTATION DISORDERS 1987-90

In other languages

URI

http://www.yso.fi/onto/mesh/D007184

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