RESUMO
INTRODUCTION: Acrokeratoelastoidosis is a rare condition characterized by bilateral multiple hyperkeratotic papules on the palms, soles, and dorsum of the hands and feet. To the best of our knowledge, only around 40 cases of acrokeratoelastoidosis have been reported worldwide, which are mostly familial. CASE PRESENTATION: We report the first case from Saudi Arabia in a 5-year-old Saudi girl of Arabian origin, who presented symptoms of acrokeratoelastoidosis with unilateral sporadic keratosis on her left hand and left foot. We also review the clinical and histopathologic features, etiology, differential diagnosis and its treatment. CONCLUSIONS: Given the rarity of acrokeratoelastoidosis, little is known about the disease. Further studies are required to understand the pathogenesis of the disease for better treatment options. Additional case reports of acrokeratoelastoidosis will help in recognizing risk factors, patient characteristics, environmental influences and possibly new etiological factors.