Zillikens D, Mehringer A, Lechner W, Burg G
Department of Dermatology, University of Würzburg, F.R.G.
Am J Dermatopathol. 1991 Feb;13(1):57-62. doi: 10.1097/00000372-199102000-00010.
Incontinentia pigmenti is a rare genodermatosis typically involving three stages: vesiculae, verrucous lesions, and hyperpigmentation. We clinically and pathologically documented a case from shortly after birth until the age of 17 years. Although the first two stages took a regular course, the third stage of the disease was characterized by hypopigmented streaks on the legs in addition to axillary hyperpigmentation. Similar hypopigmented areas were found in the patient's mother. Because pathological investigations of hypopigmented areas have been extremely rare, we performed light and electron microscopic studies and compared these with our findings in hyperpigmented regions. Light microscopy showed the hypopigmented streaks with slight epidermal atrophy and a reduced number of melanocytes and skin appendages. However, the main finding was round eosinophilic bodies in the upper dermis. Electron microscopic examination of these bodies demonstrated amorphous material that resembled colloid, suggesting degeneration of basal keratinocytes. Confirming previous reports, in hyperpigmented areas we found a reduction of pigment in those parts of the basal layer overlying melanophages located in the upper dermis.
色素失禁症是一种罕见的遗传性皮肤病，通常包括三个阶段：水疱期、疣状皮损期和色素沉着期。我们从临床和病理方面记录了一个病例，时间跨度从出生后不久直至17岁。虽然前两个阶段病程正常，但疾病的第三阶段除了腋窝色素沉着外，腿部还出现了色素减退条纹。在患者母亲身上也发现了类似的色素减退区域。由于对色素减退区域的病理研究极为罕见，我们进行了光镜和电镜研究，并将这些结果与色素沉着区域的发现进行了比较。光镜显示色素减退条纹处有轻微的表皮萎缩，黑素细胞和皮肤附属器数量减少。然而，主要发现是真皮上层有圆形嗜酸性小体。对这些小体进行电镜检查发现了类似胶体的无定形物质，提示基底角质形成细胞发生变性。与之前的报道一致，在色素沉着区域，我们发现位于真皮上层的噬黑素细胞上方的基底层部分色素减少。
