Cutaneous histopathology of Conradi-Hunermann syndrome

The histopathology of the skin in the ichthyosiform erythroderma associated with classical Conradi-Hunermann syndrome has been rarely described. Skin biopsies from 2 infants with the classical features of Conradi-Hunermann syndrome including ichthyosiform erythroderma contained hyperkeratosis, dilated ostia of pilosebaceous structures, and calcium salts in the stratum corneum detectable with special stains. An unusual feature of one case was dilatation of acrosyringeal structures. We hypothesize that dilatation of acrosyringeal structures is analogous to the plugging noted in pilosebaceous units. Taken together, the ichthyosiform erythroderma of Conradi-Hunermann has a distinctive histologic appearance.