label: palmoplantar keratoderma-XX sex reversal-predisposition to squamous cell carcinoma syndrome
label_lc: palmoplantar keratoderma-xx sex reversal-predisposition to squamous cell carcinoma syndrome
description: Palmoplantar keratoderma-XX sex reversal-predisposition to squamous cell carcinoma syndrome is characterized by sex reversal in males with a 46, XX (SRY-negative) karyotype, palmoplantar hyperkeratosis and a predisposition to squamous cell carcinoma. To date, five cases (four of whom were brothers) have been described. The etiology is unknown.