Skin fragility-woolly hair syndrome (SFWHS) is an autosomal recessive disease characterized by focal and diffuse palmoplantar keratoderma (PPK), woolly hair, recurrent bullae, and nail dystrophy, without the presence of cardiomyopathy. It is caused by a homozygous or a compound heterozygous mutation in desmoplakin, a critical component of desmosomes, which bind cells of the epidermis together.

To date, only 10 cases of SFWHS have been reported. These patients have hyperkeratotic plaques on their extremities and trunk, PPK, nail dystrophy, and woolly hair (tightly curled and slow growing) with some areas of alopecia. Recurrent infections from the recurrent bullae have been reported. Unlike other desmosome disorders, SFWHS does not improve with age. Patients with SFWHS do not suffer from cardiac abnormalities, unlike in Carvajal syndrome (CS), a similar disorder; however, overlapping cases of SFWHS and CS have been reported.