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Concept information

Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Congenital Abnormalities > Abnormalities, Multiple > Incontinentia Pigmenti
Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Genetic Diseases, Inborn > Skin Diseases, Genetic > Incontinentia Pigmenti
Skin and Connective Tissue Diseases > Skin Diseases > Skin Diseases, Genetic > Incontinentia Pigmenti
Skin and Connective Tissue Diseases > Skin Diseases > Skin Abnormalities > Incontinentia Pigmenti
Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Congenital Abnormalities > Skin Abnormalities > Incontinentia Pigmenti
Pathological Conditions, Signs and Symptoms > Pathologic Processes > Pigmentation Disorders > Incontinentia Pigmenti
Skin and Connective Tissue Diseases > Skin Diseases > Pigmentation Disorders > Incontinentia Pigmenti

Preferred term

Incontinentia Pigmenti  

Type

  • mesh:Descriptor

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.

Broader concept

Synonym(s)

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

Allowable Qualifier(s)

In other languages

  • Incontinentia pigmenti

    French

  • Dermatose pigmentaire en éclaboussures
  • Mélanose dégénérative du chorion
  • Naevus chromatophore héréditaire
  • Syndrome de Bloch-Sulzberger

URI

http://data.loterre.fr/ark:/67375/JVR-SXHL7P9J-9

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RDF/XML TURTLE JSON-LD Created 4/3/86, last modified 7/1/16