Chronic cutaneous lupus erythematosus in a linear configuration is rare,
particularly in children, demonstrating similar incidence in both genders, no
photo-sensitivity and lower probability of progression to systemic disease. We
describe the case of a 9-year-old girl who presented erythematous papules with
central atrophy on the upper and lower right limbs, asymptomatic and following
the lines of Blaschko, since age four. Histological examination showed atrophy
of the epidermis with aggression from epidermal-dermal interface and periadnexal
and perivascular lymphocytic inflammatory infiltrate. Laboratory tests showed
ANA in a titer of 1:320, in a dense and fine speckled pattern. Due to the rarity
of presentation and location of the disease, this case is reported here.