Tuesday, November 28, 2017

Negeli-Francescetti-Jadassohn syndrome.. Lack of sweating with heat intolerance. Hyperpigmentation occurs on the neck and armpits



Negeli-Francescetti-Jadassohn syndrome:
It is a rare syndrome that is inherited as an autosomal dominant, affecting young children.
Clinical manifestations:
Skin manifestations:
Hyperpigmentation occurs on the neck and armpits during the second and third years of a normal child, may become more prevalent and not preceded by any inflammation. Skin conjugation in the palms and soles is common.
General features:
- Lack of sweating with heat intolerance (common).
- Natural hair and nails.
- Teeth may be normal or damaged, with yellow discoloration in the port.
- Development of natural growth and mental.