H Syndrome: A New Genodermatosis with Distinct Features

Abstract

H syndrome (HS) is a recently described genodermatosis (inherited skin condition) characterized by progressive sclerodermatous thickening of the skin, with overlying hyperpigmentation and hypertrichosis. It primarily affects the thighs, with variable involvement of the genitalia, lower trunk, and limbs. Systemic findings of h epatosplenomegaly, h eart anomalies, h earing loss, h ypogonadism and low h eight give H syndrome its name. These authors review the cases of 10 patients (age range, …