The histopathology of the skin in the ichthyosiform erythroderma assoc
iated with classical Conradi-Hunermann syndrome has been rarely descri
bed. Skin biopsies from 2 infants with the classical features of Conra
di-Hunermann syndrome including ichthyosiform erythroderma contained h
yperkeratosis, dilated ostia of pilosebaceous structures, and calcium
salts in the stratum corneum detectable with special stains, An unusua
l feature of one case was dilatation of acrosyringeal structures. We h
ypothesize that dilatation of acrosyringeal structures is analogous to
the plugging noted in pilosebaceous units, Taken together, the ichthy
osiform erythroderma of Conradi-Hunermann has a distinctive histologic
appearance.