Boudghène-Stambouli O, Merad-Boudia A, Benkalfat M R, Khedim A
Service de Dermato-Vénéréologie, Centre Hospitalo-Universitaire, Tlemcen, Algérie.
Ann Dermatol Venereol. 1993;120(8):539-41.
We report 2 cases of acrokeratoelastoidosis of Costa observed in two sisters born of unaffected first cousins. The lesions were lenticular papules, either white or of normal skin colour, which were isolated or arranged in groups of plaques crossed by the natural furrows and located on the thenar eminences, the lateral edges of the hands and fingers and the back of the interphalangeal joints. On the feet, the lesions, located on the lateral edges, the plantar arch and the heel, were much more keratotic and appeared to be translucent. Hyperhidrosis was noted in only one of the two patients. Histological examination showed, beneath an acanthotic and hyperkeratotic epidermis, a reduced elastic network with shorter and thinner fibres in the middle dermis. These are two typical but rough cases of acrokeratoelastoidosis of Costa. The mode of transmission of this disease (autosomal recessive?) seems to be unusual compared with the data found in the literature (dominant, or sporadic cases).
我们报告了在一对表亲正常的姐妹中观察到的2例科斯塔型肢端角化性弹性组织病。皮损为扁豆状丘疹，呈白色或正常肤色，孤立存在或成群分布形成斑块，斑块被自然皮沟穿过，位于大鱼际、手部外侧边缘及手指、指间关节背面。足部皮损位于外侧边缘、足底弓及足跟，角化程度更高，呈半透明状。两名患者中仅一人有多汗症。组织学检查显示，在棘层增厚和角化过度的表皮下方，真皮中部弹性网络减少，纤维更短更细。这是2例典型但粗糙的科斯塔型肢端角化性弹性组织病病例。与文献中发现的数据（显性或散发病例）相比，这种疾病的传播方式（常染色体隐性遗传？）似乎不寻常。
