Literature DB >> 677916

Incontinentia pigmenti. Evidence for both neutrophil and lymphocyte dysfunction.

R T Jessen, D E Van Epps, J S Goodwin, J Bowerman.   

Abstract

A child with incontinentia pigmenti (Bloch-Sultzberger syndrome) had recurrent pneumococcal meningitis and pneumococcal bacteremia with associated subdural hematomas. Immunologic evaluation revealed defective neutrophil chemotaxis with normal neutrophil chemiluminescense. In addition, lymphocytes showed a depressed proliferative response to phytohemagglutinin stimulation. An immunologic defect may prove to be part of this syndrome.

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Year:  1978        PMID： 677916     DOI： 10.1001/archderm.114.8.1182

Source DB:  PubMed          Journal:  Arch Dermatol        ISSN： 0003-987X


  4 in total

1.  Studies of a family with incontinentia pigmenti variably expressed in both sexes.

Authors:  T W Kurczynski; J S Berns; W E Johnson
Journal:  J Med Genet       Date:  1982-12       Impact factor: 6.318

2.  Atypical forms of incontinentia pigmenti in male individuals result from mutations of a cytosine tract in exon 10 of NEMO (IKK-gamma).

Authors:  S Aradhya; G Courtois; A Rajkovic; R A Lewis; M Levy; A Israël; D L Nelson
Journal:  Am J Hum Genet       Date:  2001-02-08       Impact factor: 11.025

3.  X-linked dominant inherited diseases with lethality in hemizygous males.

Authors:  R Wettke-Schäfer; G Kantner
Journal:  Hum Genet       Date:  1983       Impact factor: 4.132

4.  Incontinentia pigmenti and bipolar aphthosis: an unusual combination.

Authors:  G Márquez Balbás; M A González-Enseñat; A Vicente; L Creus-Vila; J Antón; P Umbert-Millet
Journal:  ISRN Dermatol       Date:  2011-03-07
  4 in total

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