Pachydermoperiostosis is a rare osteo-cutaneous disease characterized by hypertrophy of bones and surrounding soft tissues. The cutaneous manifestations include coarsening of facial features, cutis verticis gyrata, digital clubbing, hyperhydrosis and seborrhoea. The pathogenetic mechanism of the disease is still debated, and proposed aetiological factors include genetic influences, anomalies in fibroblast activity, or alteration in peripheral blood flow. We studied a patient with the incomplete form of pachydermoperiostosis, assessing epidermal growth factor receptor (EGF-R) and sex hormone steroid receptors (SR) in the affected skin, and also evaluating the urinary excretion of EGF. The results showed high levels of nuclear steroid receptors, increased cytosolic oestrogen receptors, and no detectable progesterone and androgen cytosolic receptors. EGF-R was also undetectable, and the urinary excretion of EGF was elevated. These findings suggest that the increased tissue sensitivity to circulating sex-steroids could induce enhanced tissue EGF/transforming growth factor α (TGF-α) production and utilization. The SR-EGF-R system could therefore be involved in determining hypertrophy of the affected tissues.

Bianchi, L., Lubrano, C., Carrozzo, A.m., Iraci, S., Tomassoli, M., Spera, G., et al. (1995). Pachydermoperiostosis: Study of epidermal growth factor and steroid receptors. BRITISH JOURNAL OF DERMATOLOGY, 132(1), 128-133 [10.1111/j.1365-2133.1995.tb08638.x].

Pachydermoperiostosis: Study of epidermal growth factor and steroid receptors

BIANCHI, LUCA;CARROZZO, ANNA MARIA;NINI, GABRIELE
1995-01-01

Abstract

Pachydermoperiostosis is a rare osteo-cutaneous disease characterized by hypertrophy of bones and surrounding soft tissues. The cutaneous manifestations include coarsening of facial features, cutis verticis gyrata, digital clubbing, hyperhydrosis and seborrhoea. The pathogenetic mechanism of the disease is still debated, and proposed aetiological factors include genetic influences, anomalies in fibroblast activity, or alteration in peripheral blood flow. We studied a patient with the incomplete form of pachydermoperiostosis, assessing epidermal growth factor receptor (EGF-R) and sex hormone steroid receptors (SR) in the affected skin, and also evaluating the urinary excretion of EGF. The results showed high levels of nuclear steroid receptors, increased cytosolic oestrogen receptors, and no detectable progesterone and androgen cytosolic receptors. EGF-R was also undetectable, and the urinary excretion of EGF was elevated. These findings suggest that the increased tissue sensitivity to circulating sex-steroids could induce enhanced tissue EGF/transforming growth factor α (TGF-α) production and utilization. The SR-EGF-R system could therefore be involved in determining hypertrophy of the affected tissues.
1995
Pubblicato
Rilevanza internazionale
Articolo
Esperti anonimi
Settore MED/35 - MALATTIE CUTANEE E VENEREE
English
Con Impact Factor ISI
diethylstilbestrol; epidermal growth factor; epidermal growth factor receptor; metribolone; promegestone; steroid receptor; triamcinolone acetonide; adult; article; case report; human; hypertrophic osteoarthropathy; male; priority journal; Adult; Case Report; Epidermal Growth Factor; Human; Male; Osteoarthropathy, Primary Hypertrophic; Protein Binding; Receptor, Epidermal Growth Factor; Receptors, Androgen; Receptors, Estradiol; Receptors, Progesterone; Receptors, Steroid
Bianchi, L., Lubrano, C., Carrozzo, A.m., Iraci, S., Tomassoli, M., Spera, G., et al. (1995). Pachydermoperiostosis: Study of epidermal growth factor and steroid receptors. BRITISH JOURNAL OF DERMATOLOGY, 132(1), 128-133 [10.1111/j.1365-2133.1995.tb08638.x].
Bianchi, L; Lubrano, C; Carrozzo, Am; Iraci, S; Tomassoli, M; Spera, G; Nini, G
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2108/56985
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