Abstract
Skin fragility, palmoplantar keratoderma, and characteristic woolly hair are defining features of skin fragility-woolly hair syndrome (SFWHS), an extremely rare inherited disorder. This condition is classified within the group of desmosomal disorders, which are associated with compromised structural integrity of the skin and hair. Clinically, affected individuals often present with hair fragility, fragile skin, recurrent erosions, nail abnormalities, and varying degrees of palmoplantar thickening beginning in early childhood. In this case report, we describe a patient with this extremely rare syndrome.