PACHYDERMOPERIOSTOSIS - STUDY OF EPIDERMAL GROWTH-FACTOR AND STEROID-RECEPTORS

Pachydermoperiostosis is a rare osteo-cutaneous disease characterized by hypertrophy of bones and surrounding soft tissues. The cutaneous ma nifestations include coarsening of facial features, cutis verticis gyr ata, digital clubbing, hyperhidrosis and seborrhoea. The pathogenetic mechanism of the disease is still debated, and proposed aetiological f actors include genetic influences, anomalies in fibroblast activity, o r alteration in peripheral blood now. We studied a patient with the in complete form of pachydermoperiostosis, assessing epidermal growth fac tor receptor (EGF-R) and sex hormone steroid receptors (SR) in the aff ected skin, and also evaluating the urinary excretion of EGF. The resu lts showed high levels of nuclear steroid receptors, increased cytosol ic oestrogen receptors, and no detectable progesterone and androgen cy tosolic receptors. EGF-R was also undetectable, and the urinary excret ion of EGF was elevated. These findings suggest that the increased tis sue sensitivity to circulating sex-steroids could induce enhanced tiss ue EGF/transforming growth factor ct (TGF-m) production and utilizatio n. The SR-EGF-R system could therefore be involved in determining hype rtrophy of the affected tissues.