Benign atrophic papulosis (Köhlmeier-Degos disease): the wedge-shaped dermal necrosis can resolve with time

J Eur Acad Dermatol Venereol. 2017 Oct;31(10):1753-1756. doi: 10.1111/jdv.14355. Epub 2017 Jun 20.


Background: Atrophic papulosis is a rare thrombo-occlusive disease, characterized by the appearance of multiple atrophic porcelain-white skin papules, with a surrounding erythematous rim, which are histologically consisting of wedge-shaped necrosis of the dermis.

Objective: It consists of two variants: (i) the benign atrophic papulosis (BAP) only involving the skin and (ii) the malignant atrophic papulosis (MAP) also involving several internal organs with a cumulative five-year survival rate of approx. 55%. While the probability of only having a BAP at onset is approximately 70%, increasing to 97% after 7 years of monosymptomatic cutaneous course, no close long-term follow-up of the development of the skin lesions has been reported.

Methods: We present a precise visual documentation of the evolution of the disseminated skin lesions in a female patient with BAP spanning over two decades.

Results: A considerable improvement and/or clinical resolution of the majority of the lesions disputing the scarring character of the atrophic porcelain-white skin papules has been detected.

Conclusion: BAP not only exhibits an excellent prognosis, but resolution of lesions can also occur after a considerable period of time.

Publication types

  • Case Reports

MeSH terms

  • Biopsy
  • Dermatologic Agents / therapeutic use
  • Female
  • Humans
  • Malignant Atrophic Papulosis / drug therapy
  • Malignant Atrophic Papulosis / pathology*
  • Malignant Atrophic Papulosis / physiopathology
  • Middle Aged
  • Necrosis
  • Skin / pathology*
  • Survival Rate


  • Dermatologic Agents