Woolly Hair-Skin Fragility Syndrome, also known as skin fragility-woolly hair syndrome, is related to arrhythmogenic right ventricular dysplasia, familial, 8 and cardiomyopathy, dilated, with woolly hair and keratoderma. An important gene associated with Woolly Hair-Skin Fragility Syndrome is TUFT1 (Tuftelin 1). Affiliated tissues include skin.