Peris K, Salvati E F, Torlone G, Chimenti S
Department of Dermatology, University of L'Aquila, Italy.
Br J Dermatol. 1995 Apr;132(4):617-20. doi: 10.1111/j.1365-2133.1995.tb08721.x.
Keratoderma hereditarium mutilans, or Vohwinkel's syndrome, is a rare cutaneous disorder which is characterized by thickening of the palms, soles and dorsa of the hands and feet, and by ainhum-like constrictions of the fingers. We report a clinically typical case of Vohwinkel's syndrome in a 28-year-old, deaf-mute, woman. The patient presented with keratotic palms and soles which had a 'honeycomb' appearance, starfish-like keratoses on the dorsa of the hands, and pseudoainhum of the digits. Osteoporotic changes were present distal to the constricting bands. Successful treatment with retinoids has been reported recently, and the hyperkeratosis and constricting bands in our patient improved on therapy with etretinate.
遗传性残毁性角皮病，即Vohwinkel综合征，是一种罕见的皮肤疾病，其特征为手掌、足底及手足背部增厚，以及手指出现类似自发性断指症的缩窄。我们报告一例28岁聋哑女性的临床典型Vohwinkel综合征病例。患者表现为掌跖角化，呈“蜂窝状”外观，手背有海星状角化病，手指有假性自发性断指症。缩窄带远端存在骨质疏松改变。最近有报道称维甲酸治疗成功，我们的患者经依曲替酯治疗后，角化过度和缩窄带有所改善。
