Literature DB >> 7451148

[Netherton syndrome: weakened immunity, generalized verrucosis and carcinogenesis].

H Hintner, E Jaschke, P Fritsch.   

Abstract

Two patients with Netherton's syndrome are presented who, in addition to the classical triad of trichorrhexis invaginata, ichthyosis linearis circumflexa and atopy, exhibit signs of reduced cellular immunity (negative skin tests to a battery of microbial antigens, impaired stimulation of lymphocytes with various mitogens and widespread viral warts resembling epidermodysplasia verruciformis). One patient had a squamous cell carcinoma possibly arising from a viral wart. It is suggested that Netherton's syndrome is part of the group of immunodeficiency syndromes with atopy-like symptoms.

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Year:  1980        PMID: 7451148

Source DB:  PubMed          Journal:  Hautarzt        ISSN: 0017-8470            Impact factor:   0.751


  6 in total

Review 1.  Ichthyosis update: towards a function-driven model of pathogenesis of the disorders of cornification and the role of corneocyte proteins in these disorders.

Authors:  Matthias Schmuth; Robert Gruber; Peter M Elias; Mary L Williams
Journal:  Adv Dermatol       Date:  2007

Review 2.  Etretinate therapy in children with severe keratinization defects.

Authors:  H Traupe; R Happle
Journal:  Eur J Pediatr       Date:  1985-01       Impact factor: 3.183

Review 3.  Warts and all: human papillomavirus in primary immunodeficiencies.

Authors:  Jennifer W Leiding; Steven M Holland
Journal:  J Allergy Clin Immunol       Date:  2012-10-01       Impact factor: 10.793

4.  Netherton's syndrome.

Authors:  G M Murphy; W A Griffiths; K Grice
Journal:  J R Soc Med       Date:  1989-11       Impact factor: 18.000

5.  Netherton syndrome - Why ENT surgeons should be aware of this rare disease - report of a case.

Authors:  Kornelia Ec Wirsching; Julia Heinlin; Holger G Gassner
Journal:  BMC Ear Nose Throat Disord       Date:  2013-07-06

6.  Genetic activation of Nrf2 reduces cutaneous symptoms in a murine model of Netherton syndrome.

Authors:  Sukalp Muzumdar; Michael Koch; Hayley Hiebert; Andreas Bapst; Alessia Gravina; Wilhelm Bloch; Hans-Dietmar Beer; Sabine Werner; Matthias Schäfer
Journal:  Dis Model Mech       Date:  2020-06-01       Impact factor: 5.758

  6 in total

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