Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, Theodore Fontane Medical University of Brandenburg, Dessau, Germany.
Freiburg Veins Center, Freiburg, Germany.
J Eur Acad Dermatol Venereol. 2017 Oct;31(10):1753-1756. doi: 10.1111/jdv.14355. Epub 2017 Jun 20.
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.
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.
We present a precise visual documentation of the evolution of the disseminated skin lesions in a female patient with BAP spanning over two decades.
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.
BAP not only exhibits an excellent prognosis, but resolution of lesions can also occur after a considerable period of time.
萎缩性丘疹病是一种罕见的血栓闭塞性疾病，其特征是出现多个萎缩性瓷白色皮肤丘疹，周围有红斑环，组织学上由真皮楔形坏死组成。
它由两种变异型组成：（i）良性萎缩性丘疹病（BAP）仅累及皮肤，（ii）恶性萎缩性丘疹病（MAP）还累及多个内脏器官，五年累积生存率约为 55%。虽然发病时仅出现 BAP 的概率约为 70%，但在 7 年的单一皮肤症状病程后增加到 97%，但没有关于皮肤病变发展的密切长期随访报道。
我们展示了一位患有 BAP 的女性患者的弥漫性皮肤病变长达二十年的精确视觉记录。
大多数病变都有明显改善和/或临床消退，这与萎缩性瓷白色皮肤丘疹的瘢痕特征相矛盾。
BAP 不仅表现出极好的预后，而且在相当长的时间后也可以消退。
