Brusasco A, Cambiaghi S, Tadini G, Berti E, Caputo R
Centre for Inherited Cutaneous Diseases, Institute for Dermatological Sciences, IRCCS, Ospedale Maggiore, University of Milan, Via Pace 9, 20122 Milan, Italy.
Br J Dermatol. 1998 Nov;139(5):893-6. doi: 10.1046/j.1365-2133.1998.02521.x.
We present an unusual new clinical feature which developed in a patient with congenital reticular ichthyosiform erythroderma. This rare ichthyotic disorder is characterized by erythematous ichthyotic skin surrounding slowly enlarging areas of normal skin, and by a pathognomonic ultrastructural pattern, namely perinuclear deposits of a filamentous material in vacuolized keratinocytes. At the age of 18 years, a 23-year-old woman developed several irregular hyperpigmented macules on her limbs, which were almost black in colour. These lesions have not been observed in the other patients affected by the disease nor, to our knowledge, in other ichthyotic disorders. Electron microscopy and immunohistochemistry demonstrated that the lesions were strictly related to the ichthyotic skin and that their dark colour was especially due to melanosome accumulation in activated dendritic melanocytes. An unusual postinflammatory hyperpigmentation, in which the lack of pigment deposition in the keratinocytes is due to a transfer defect in pathological cells, is hypothesized. A characteristic hyperplastic stimulation of the epidermis is also taken into consideration to explain the lack of a similar picture in other erythrodermic ichthyotic disorders with a continuous inflammatory process.
我们报告了一名先天性网状鱼鳞病样红皮病患者出现的一种罕见的新临床特征。这种罕见的鱼鳞病性疾病的特点是，正常皮肤缓慢扩大区域周围出现红斑性鱼鳞病样皮肤，以及一种具有诊断意义的超微结构模式，即在空泡化角质形成细胞的核周有丝状物质沉积。一名23岁女性在18岁时四肢出现了几个不规则的色素沉着过度斑，颜色几乎呈黑色。在其他患该病的患者中未观察到这些病变，据我们所知，在其他鱼鳞病性疾病中也未观察到。电子显微镜和免疫组织化学显示，这些病变与鱼鳞病样皮肤密切相关，其深色尤其归因于活化的树突状黑素细胞中黑素体的积累。推测这是一种不寻常的炎症后色素沉着过度，其中角质形成细胞中缺乏色素沉积是由于病理细胞中的转运缺陷所致。还考虑到表皮的一种特征性增生刺激，以解释在其他具有持续炎症过程的红皮病性鱼鳞病性疾病中为何没有类似表现。
