CUTANEOUS HISTOPATHOLOGY OF CONRADI-HUNERMANN SYNDROME

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.