Erkek Emel, Koçak Mukadder, Bozdoğan Onder, Atasoy Pinar, Birol Ahu
Department of Dermatology, Faculty of Medicine, Kirikkale University, Kirikkale, Turkey.
Pediatr Dermatol. 2004 Mar-Apr;21(2):128-30. doi: 10.1111/j.0736-8046.2004.21208.x.
Acrokeratoelastoidosis and focal acral hyperkeratosis share similar clinical features and identical histologic epidermal alterations. These disorders are distinguished solely on the basis of the absence of elastorrhexis in the latter. We present a case of focal acral hyperkeratosis in a 9-year-old girl. The lesions consisted of translucent polygonal papules clustered on the thenar regions of the palms and over the metacarpophalangeal and interphalangeal joints. Histopathologic examination revealed orthohyperkeratosis within focal clavus-like depressions of the epidermis and prominent hypergranulosis. There was no evidence of alterations in elastic tissue. The clinicopathologic distinction between focal acral hyperkeratosis and acrokeratoelastoidosis is blurred. There is enough evidence to consider the former as a histologic variant of Costa acrokeratoelastoidosis syndrome, and a better nomenclature for this disorder would be "acrokeratoelastoidosis without elastorrhexis."
肢端角化性弹性组织变性病和局限性肢端角化过度症具有相似的临床特征和相同的组织学表皮改变。这两种疾病仅根据后者不存在弹性纤维离解来区分。我们报告一例9岁女孩的局限性肢端角化过度症。病变由聚集在手掌大鱼际区域以及掌指关节和指间关节处的半透明多边形丘疹组成。组织病理学检查显示表皮局灶性鸡眼样凹陷内有正角化过度和明显的颗粒层增厚。没有弹性组织改变的证据。局限性肢端角化过度症和肢端角化性弹性组织变性病之间的临床病理区别并不明确。有足够的证据将前者视为科斯塔肢端角化性弹性组织变性综合征的一种组织学变体，这种疾病更好的命名应为“无弹性纤维离解的肢端角化性弹性组织变性病”。
