Section of Dermatology, DISSAL, and Institute of Pathology, University of Genoa, Genoa, Italy.
Br J Dermatol. 2012 Sep;167(3):575-82. doi: 10.1111/j.1365-2133.2012.11003.x. Epub 2012 Jul 19.
Aquagenic keratoderma is an uncommon condition that occurs after brief water exposure. An association with cystic fibrosis has been suggested. Histopathology is considered to be nonspecific.
To describe the microscopic findings in seven of 12 new patients and compare the histopathological results of the lesions which appeared on the palmar skin after immersion into water with normal skin.
Nine female and three male patients (mean age 27 years) were collected prospectively and evaluated for common demographic, clinical and histopathological features.
Lesions were located on only the palms in seven patients; the soles were involved in two patients; and one patient had involvement of the dorsal aspect of the hands. One patient had a similar family history. None of the patients reported associated conditions. Genetic studies revealed heterozygosis for mutation in the cystic fibrosis gene in two patients. The most specific histopathological findings were: orthohyperkeratosis with increased thickness and abnormal staining of the stratum corneum; dilated acrosyringia and dermal eccrine ducts with hyperplasia of eccrine glands, clear cell change and vacuolation; increased capillaries around and adjacent to the eccrine glands. A skin biopsy taken after restoration of normal skin with drying revealed a normal stratum corneum with a physiological uniform stain and normal thickness without further evidence of dilation of acrosyringia or dermal eccrine ducts. Incipient dilation of the secretory and ductal structures was also observed in a transitional area between the involved and the clinically normal skin of the palms.
Aquagenic keratoderma may be associated with a heterozygous mutation in the cystic fibrosis gene. Although the diagnosis is a clinical one, histopathology is useful and may reveal some characteristic diagnostic clues. Aquagenic pseudokeratoderma seems to be a more appropriate term to name it.
水变性角化病是一种罕见的疾病，发生在短暂的水暴露后。有人认为它与囊性纤维化有关。组织病理学被认为是非特异性的。
描述 12 例新患者中的 7 例的微观发现，并比较在水中浸泡后出现在手掌皮肤上的病变与正常皮肤的组织病理学结果。
前瞻性收集 9 名女性和 3 名男性患者（平均年龄 27 岁），并评估常见的人口统计学、临床和组织病理学特征。
7 例患者仅手掌受累，2 例患者足底受累，1 例患者手掌背侧受累。1 例患者有类似的家族史。无患者报告相关疾病。遗传研究显示，2 例患者囊性纤维化基因杂合突变。最特异的组织病理学发现为：角质层过度角化，角质层厚度增加，异常染色；顶泌汗腺和真皮小汗腺扩张，大汗腺增生，透明细胞改变和空泡化；周围和相邻的小汗腺毛细血管增加。在干燥恢复正常皮肤后进行的皮肤活检显示，正常的角质层具有生理均匀的染色和正常的厚度，没有进一步扩张的顶泌汗腺或真皮小汗腺的证据。在手掌受累和临床正常皮肤的过渡区域，也观察到分泌和导管结构的早期扩张。
水变性角化病可能与囊性纤维化基因的杂合突变有关。尽管诊断是临床诊断，但组织病理学是有用的，可能会揭示一些特征性的诊断线索。水变性假性角化病似乎是一个更合适的术语来命名它。
