Literature DB >> 28544089

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

C C Zouboulis1, A Theodoridis1,2, M Brunner1, C M Magro3.   

Abstract

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.
© 2017 European Academy of Dermatology and Venereology.

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Year:  2017        PMID: 28544089     DOI: 10.1111/jdv.14355

Source DB:  PubMed          Journal:  J Eur Acad Dermatol Venereol        ISSN: 0926-9959            Impact factor:   6.166


  2 in total

1.  [The benign form of malignant atrophic papulosis (Köhlmeier-Degos disease)].

Authors:  Carolin Haas; Tanja von Braunmühl; Thomas Herzinger
Journal:  Hautarzt       Date:  2018-10       Impact factor: 0.751

2.  A Case of Benign Atrophic Papulosis in a Young Male.

Authors:  Lingyi Lu; Bingjiang Lin; Ru Dai; Xin Fan; Yingzhe Yu; Ying Qi; Xiaoxia Zhu; Ping Zhou
Journal:  Ann Dermatol       Date:  2022-08       Impact factor: 0.722

  2 in total

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