Case report 106

Abstract

Fig. I. A photograph of the dorsum of both hands shows the skin to be smooth and shining. Thickened, horny plates and warty nodules are observed HistoryThis 16-year-old white male presented in the Dermatology Clinic because of erythroderma and hyperkeratosis. The condition had been present since birth, consisting of a dull erythema, generalized from the beginning and most intense in the flexures. The skin was smooth, shining, and thickened. Horny plates and warty nodules, both irregular and linear, were observed. These were particularly present at the sides and back of the neck, around the umbilicus, on the areola of the nipples and the limb flexures, including the hands and feet (Fig. 1). The nails exhibited progressive hour-glass deformity without associated clubbing.In the past history, generalized edema had been observed shortly after birth, subsiding spontaneously in a few days. At the age of 10 years, the patient started to develop bilateral ectropion and because of infection the left eye was enucleated at the age of 12 years.The remainder of the history was not contributory except for the information that the parents of the patient were first co usins.Roentgenograms of the hands ( Figs. 2A and B) and feet were obtained. Fig. 2A and B. Posteroanterior roentgenograms of both hands demonstrate resorption of the terminal tufts of most of the distal phalanges. The surrounding soft tissues are noted to be thickened and the normal fissures of the skin appear to be randomly dispersed (the changes in the feet were similar but less severe)