Hailey-Hailey disease is a recurrent, autosomal dominant vesiculobullous de
rmatotis with a predilection for intertrigenous areas. We report what we be
lieve to be the first case of squamous cell carcinoma arising de novo in a
skin lesion of Hailey-Hailey disease. The occurrence of malignant neoplasms
arising in the skin lesions of Hailey-Hailey disease and other acantholyti
c dermatoses is reviewed.