A PARTICULAR SUBTYPE OF ICHTHYOSIS CONGENITA TYPE-III - CLINICAL, LIGHT, AND ELECTRON-MICROSCOPIC FEATURES

We report the case of a second patient with the extraordinary ultrastr uctural findings of vacuolated structures intermingled with membranes in the perinuclear part of the upper epidermal cells. Clinical, light microscopic, and electron microscopic features of this particular pres entation of ichthyosis congenita type III have already been presented by K. M. Niemi and L. Kanerva in 1989. Although our patient has more o r less the same light and electron microscopic findings, the clinical picture is more severe. The patient was born as a collodion baby. Late r, he showed signs of generalized severe involvement with large scales , erythrodermia, and itching. Successful therapy with retinoids result ed in complete removal of the hyperkeratosis but left the striking ret iculate skin pattern. Noting the heterogeneous clinical presentation, the specific electron microscopic findings are diagnostic. No biochemi cal data on this disease are known.