Brunauer-Fuhs-Siemens palmoplantar keratoderma: A rare, striate type of focal palmoplantar keratoderma

Brunauer-Fuhs-Siemens palmoplantar keratoderma, commonly known as striate palmoplantar keratoderma, is a rare, autosomally inherited disease of linear hyperkeratosis in which patient usually presents with conspicuous longitudinal hyperkeratosis on volar surface of hands and feet. Mutations in 3 genes namely desmoglein 1, desmoplakin and keratin 1, have been identified and held responsible for this type of keratoderma which can efficiently lead to the structural defects in desmosomal plaque proteins manifesting as mechanical weakness in the pressure areas of palm and soles. Apart from the cutaneous features, woolly hair and cardiomyopathy may also be assosiated with striate palmoplantar keratoderma. We herein report a unique case of striate palmoplantar keratoderma in a 18 years old male having linear hyperkeratosis over dorsal surface of hands and feet alongwith nummular keratoderma since chilhood.