Format

Send to

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

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

Author information

1
Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, Theodore Fontane Medical University of Brandenburg, Dessau, Germany.
2
Freiburg Veins Center, Freiburg, Germany.
3
Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY, USA.

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.

PMID:
28544089
DOI:
10.1111/jdv.14355
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Wiley
Loading ...
Support Center