THE NAGELI-FRANCESCHETTI-JADASSOHN SYNDROME - A HEREDITARY ECTODERMALDEFECT LEADING TO COLLOID-AMYLOID FORMATION IN THE DERMIS

Light- and electron-microscopical examination of 4 skin biopsies from 2 members of the initially described family with Nageli-Franceschetti- Jadassohn syndrome revealed that the already reported pigment incontin ence is accompanied by varying amounts of colloid-amyloid bodies locat ed in the superficial dermis. Occasionally, such bodies could also be seen around sweat glands in the reticular dermis. These findings indic ate that cutaneous colloid-amyloid formation could be a pathogenic fac tor in the phenotypic expression of this autosomal dominant syndrome.