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DeCS
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Descriptor English:
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Incontinentia Pigmenti
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Descriptor Spanish:
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Incontinencia Pigmentaria
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Descriptor Portuguese:
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Incontinência Pigmentar
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Synonyms English:
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Bloch Sulzberger Syndrome
Bloch-Siemens Syndrome
Bloch-Siemens-Sulzberger Syndrome
Bloch-Sulzberger Syndrome
Syndrome, Bloch-Sulzberger
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Tree Number:
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C16.131.077.445
C16.131.831.580
C16.320.850.420
C17.800.621.497
C17.800.804.580
C17.800.827.420
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Definition English:
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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. |
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Indexing Annotation English:
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multiple skin & other abnorm; do not confuse with INCONTINENTIA PIGMENTI ACHROMIANS see PIGMENTATION DISORDERS
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History Note English:
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91(87); was see under PIGMENTATION DISORDERS 1987-90
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Allowable Qualifiers English:
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Record Number:
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19206
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Unique Identifier:
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D007184
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Occurrence in VHL:
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Similar:
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DeCS
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