A 12-year-old girl, with atopic dermatitis since childhood, was referred for an acquired and painful transgradient palmoplantar keratoderma (PPK), thought to be inherited. She had no relevant family history. She presented with palmar pruritus for three months, which had been diagnosed as atopic eczema. Despite topical corticosteroid treatment, the cutaneous lesions worsened. Additionally, she had a 6 kg weight loss. Physical examination showed hyperkeratotic, fissured, and painful plaques on the palms (Figures 1,2) and the soles associated with subungual hyperkeratosis.