Progressive symmetrical erythrokeratodermia (PSEK), also known as Gottron’s disease, is a rare autosomal dominant genodermatosis with variable penetrance presenting as sharply defined, erythematous and hyperkeratotic plaques symmetrically over the limbs and buttocks. This is a report of two siblings presenting with these lesoins which started at the age of 5 years in the male and 3 years in the female born out of third degree consanguineous marriage. Both were treated with 0.5mg/kg oral isotretinoin and moisturisers containing urea which showed drastic improvement in two months. Histopathology showed marked ortho hyperkeratosis and focal irregular areas of acanthosis and focal papillomatosis. It’s important to rule out psoriasis which is it’s closest differential diagnosis. Lesions progress during childhood and stabilise after puberty