[Type I pachyonychia congenita (Jadarssohn-Lewandowsky)].

Abstract:

BACKGROUND:Pachyonychia congenita is considered to be a genodermatosis of autosomal inheritance. It is characterized by nail hypertrophy, shortly present after birth. Later on follicular keratosis of the extremities and hyperkeratosis of palms and soles can be found. HISTORY AND CLINICAL FINDINGS:We report a child with pachyonychia congenita type-I (Jadassohn-Lewandowsky). Shortly after birth nail hypertrophy of all finger- and toenails and leukoplakia of the palate and tongue were found. At the age of 3 years follicular keratosis of the extremities and plantar bullae could be found additionally. CONCLUSION:The underlying disturbance is a mutation within genes for keratin 6, 16 and 17 which leads to formation of abnormal tonofilaments. In adult patients retinoids can be used for symptomatic treatment especially of the palmoplantar keratosis.

journal_name

Klin Padiatr

journal_title

Klinische Padiatrie

authors

Wimmershoff MB,Stolz W,Schiffner R,Landthaler M

doi

10.1055/s-2008-1043783

keywords:

subject

Has Abstract

pub_date

1999-05-01 00:00:00

pages

179-83

issue

3

eissn

0300-8630

issn

1439-3824

journal_volume

211

pub_type

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  • [Influence of nutrition on the cellular defence mechanism (phagocytosis) of preterm and underweight babies during the first six weeks of life (author's transl)].

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  • What are Palliative Care Physicians for Adults Taught on Palliative Care for Children? Paediatric Aspects in Palliative Care Curricula for Adults.

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  • 15 parameters of coagulation and fibrinolysis in children with type I diabetes mellitus (onset period).

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