Clinical case of a very rare form of keratodermia in a child

Cases of keratoderma are the most common among genodermatoses and take the second place after a group of ichthyosis. The frequency of occurrence of keratodermia in the world, including Russia, is from 1:300 to 1:15000. There is a very rare form of diffuse keratoderma: Olmsted Syndrome, which in 2009 is described in 10 sources of scientific literature. The defeat not only affects the palms and soles, and skin around the orifices, forms a flexion deformity and contracture fingers, until spontaneous amputation. Some observations noted the presence of follicular keratosis of the extremities, leukokeratosis on the face and language, pererastayut joints, alopecia, absence of premolars. We present a clinical case of a patient with this pathology.