外生殖器部位棘层松解性皮病

Acantholytic dermatosis of the vulva

报告1例外生殖部位棘层松解性皮病。患者女,54岁。外阴灰白色、肤色丘疹伴瘙痒8个月。皮肤科检查:双侧大阴唇可见暗红色水肿性红斑,边界清楚,红斑上散在线状排列的米粒至黄豆大肤色、苍白色圆形扁平丘疹,表面光滑,质地较硬;部分丘疹融合形成斑块,呈苔藓样变,散在抓痕、糜烂。皮损组织病理检查:表皮灶性角化过度、角化不全,棘层肥厚,棘细胞松解,角化不良,表皮下血管增生,淋巴细胞浸润。直接免疫荧光检查示阴性。诊断:外生殖器部位棘层松解性皮病。

A case of acantholytic dermatosis of the vulva is reported.A 54-year-old woman presented with gray,white to skin-colored,pruritic papules,in the region of the vulva,for eight months.Clinical examination showed dark-red,edematous,sharply demarcated plaques,on both sides of the labia majora.Multiple rice-sized to soybean-sized,whitish papules were noted on the surface of the erythematous plaques on the labia majora.Histopathological examination showed acanthosis of the epidermis with acantholysis and dyskeratosis.A patchy lymphocytic infiltrate and increased numbers of small vessels were noted in the dermis.Direct immunofluorescence study showed a negative finding.The diagnosis of acantholytie acanthoma of the vulva was made.