恶性萎缩性丘疹病并肠穿孔、腹膜炎1例

A Case of Malignant Atrophic Papulosis with Intestinal Perforation and Peritonitis

患者男,54岁,躯干、四肢红色丘疹3年,反复腹痛5个月,诊断为"腹膜炎",影像学检查示:肠穿孔。皮肤镜示:皮损周边为主树枝状血管、袢状血管和不规则血管,中央形状不规则白色无结构区。皮损组织病理示:表皮萎缩,基底细胞液化变性,真皮浅层部分胶原楔形透明变性,血管周围少数淋巴细胞浸润,胶原排列紊乱;特殊染色:AB-PAS(+)。诊断为恶性萎缩性丘疹病。

A 54-year-old male present red papules on the trunk and limbs for 3 years,with repeated abdominal pain for 5 months,imaging examination revealed intestinal perforation,and was diagnosed as"peritonitis".Dermoscopic features were peripheral arborizing vessels,looped vessels and linear irregular vessels with central white structureless area.Histological features of skin lesion were epidermal atrophy,basal cell liquefaction,wedge-shaped collagen degeneration in upper dermis,spares perivascular lymphocytic infiltration,disarrangement of collagen fibers,AB-PAS stain was postive.The diagnosis of malignant atrophic papulosis was made.