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Malignant atrophic papulosis Kohlmeier-Degos is a very rare disease of unknown etiology. It affects the skin with disseminated erythematous papules on the trunk and extremities, which heal with atrophic porcelain-white scars with a telangiectatic rim. In the majority of patients, extracutaneous manifestations, mostly of the gastrointestinal tract or the central nervous system, develop after a variable period of time. We report a 21-year-old female patient in whom the diagnosis of malignant atrophic papulosis was based on the distinct clinical appearance and histopathologic alterations. Laboratory investigations, including haemostasis, autoimmunity, and vasculitis specific parameters were all negative, as was direct immunfluorescence. The patient has had no extracutaneous manifestations during a 4-year-disease duration. There is no established effective treatment. Neither topical corticosteroids nor pimecrolimus were of any benefit in our patient. This patient may represent one of the rare cases of a benign variant of malignant atrophic papulosis limited to the skin.

Papulosis maligna atrophicans Köhlmeier-Degos ohne Systembefall bei einer 21-jährigen Patientin