On the basis of 106 patients with malignant atrophic papulosis in the literature, including one case of our own, the clinical, histopathological and nosological features of this disease are reviewed. Histopathologically, the typical papules with central porcelain-white atrophy show broad wedge-shaped necrosis of the connective tissue due to thrombotic occlusions of small vessels in the corium. The disease leads to death in 50% of these cases, mostly due to involvement of the gut and the central nervous system. Malignant atrophic papulosis is the prototype of a disease, in which skin lesions are the primary sign of a systemic disorder demanding the interdisciplinary cooperation of various specialists.