Keratoderma hereditarium mutilans (Vohwinkel syndrome).

ABSTRACT

Keratoderma hereditarium mutilans, or Vohwinkel syndrome, is a very rare genetic skin condition which causes palmoplantar hyperkeratosis and constricting rings of the fingers and toes. Approximately 50 cases have been reported in the literature with only three having been managed surgically. All three had a high rate of recurrence and unfavourable results in the long term. We report two more cases managed surgically with a follow up of 5 and 8 years respectively. Our experience suggests that the use of full thickness grafts to line the released contractures does not work in the long term as the grafts become raised and painful, requiring multiple revisions. Surgical correction was easy to achieve but difficult to maintain and achieved poor outcomes in general. We therefore feel that the indication for surgical treatment should be a neurovascular compromise.