皮损形态类似于色素失调症的局灶性真皮发育不全

Focal dermal hypoplasia presenting as incontinentia pigmenti:a case report

报告1例皮损形态类似于色素失调症的局灶性真皮发育不全。患者女,30岁。因全身条纹状和不规则形的色素斑近30年就诊。皮损沿Blaschko线分布,主要由色素沉着斑、色素减退斑和萎缩斑等组成。此外,患者还有右足第3和第4趾的并趾畸形。组织病理检查:真皮发育不良,明显变薄,皮下脂肪组织上移。弹性纤维染色显示真皮上层的弹性纤维明显减少,而真皮中、下层的弹性纤维却基本正常。临床上主要需与色素失调症鉴别。

A case of focal dermal hypoplasia presenting as incontinentia pigmenti is reported. A 30-year-old woman presented with hyperpigmentation, hypopigmentation and atrophy macules all over the body since birth. The lesions follow Blaschko＇s lines. She also had syndactylies of the third and fourth toes of the right foot since birth. Biopsy specimens showed dermal hypoplasia, attenuation of the dermis, and upward extension of the fat tissue. Verhoeff and acid orcein-Giemsa stains showed a remarkable diminution of fine elastic fibers from the papillary to upper reticular dermis, whereas those of the mid dermis were intact. Differential diagnosis must be made with incontinentia pigmenti.