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

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: a) the benign atrophic papulosis (BAP) only involving the skin and b) the malignant atrophic papulosis (MAP) also involving several internal organs with a cumulative 5-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.

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