Unit of Plastic Surgery, University of Padua, Padua, Italy.
Int J Dermatol. 2010 Jan;49(1):79-82. doi: 10.1111/j.1365-4632.2009.04267.x.
Vohwinkel syndrome or keratoderma hereditaria mutilans is a rare autosomal dominant palmoplantar keratosis which manifests in infants and becomes more evident in adulthood. Patients with this mutation present hyperkeratosis of the palms and soles, constricting bands of the digits, usually at the fifth, and starfish-shaped hyperkeratosis on the dorsal aspects of the hands and feet. The disease mostly occurs in white women, where constricting fibrous bands appear on the digits and can lead to progressive strangulation and auto-amputation (pseudo-ainhum).
The treatment of this keratoderma is very difficult and tends to be symptomatic: topical keratolytics and systemic retinoids have been used to treat hyperkeratosis, but without consistent results. Reconstructive surgery is utilized for the treatment of pseudo-ainhum.
In this study, we present an additional case of Vohwinkel syndrome in which constrictive bands of the fifth digit in the left hand were treated with a cross finger flap, with a favorable outcome after 18 months of follow-up.
Vohwinkel 综合征或遗传性进行性肢端角化症是一种罕见的常染色体显性掌跖角化病，在婴儿期发病，成年后更为明显。患有这种突变的患者手掌和脚底出现过度角化，手指出现缩窄带，通常在第五指，手和脚的背侧出现海星状过度角化。这种疾病主要发生在白人女性中，手指上会出现缩窄的纤维带，可导致进行性绞窄和自截（假性拇指离断）。
这种角化病的治疗非常困难，往往是对症治疗：局部角质松解剂和全身性维 A 酸类药物已被用于治疗过度角化，但结果不一致。重建手术用于治疗假性拇指离断。
在本研究中，我们报告了一例额外的 Vohwinkel 综合征病例，左手第五指的缩窄带采用交叉指皮瓣治疗，18 个月随访后结果良好。
