We report what we believe to be a novel skin manifestation of dioxin intoxication. A 30-year-old woman with 2,3,7, 8-tetrachlorodibenzo-p-dioxin levels of 144,000 pg g-1 blood fat presented with severe chloracne that affected the entire integument. She also exhibited acral granuloma annulare-like lesions and distal onycholysis and, at a later time point, showed signs of hypertrichosis, as well as brownish-grey hyperpigmentation of the face. In addition, she developed punctate keratoderma-like lesions on the palms and soles. These lesions were negative for human papillomavirus and histologically characterized by cone-shaped hyperkeratoses invaginating, but not penetrating, into the dermis. Squamous syringometaplasia of the eccrine glands was observed in the immediate vicinity of these lesions. Both clinically and histologically these alterations are essentially indistinguishable from what is described as keratosis punctata palmaris et plantaris (KPPP). Although a fortuitous coincidence of chloracne and KPPP cannot be formally excluded, the possibility exists that in our patient toxic levels of dioxin were causally involved in this disorder of keratinization.