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Benign atrophic papulosis (Köhlmeier–Degos disease): the wedge‐shaped dermal necrosis can resolve with time
Author(s) -
Zouboulis C.C.,
Theodoridis A.,
Brunner M.,
Magro C.M.
Publication year - 2017
Publication title -
journal of the european academy of dermatology and venereology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.655
H-Index - 107
eISSN - 1468-3083
pISSN - 0926-9959
DOI - 10.1111/jdv.14355
Subject(s) - medicine , dermis , dermatology , necrosis , pathology
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.