Long-Term Surgical Success in Treating Vohwinkel Syndrome: A Case Report.

CASE: Vohwinkel syndrome is a rare, inherited condition marked by defective keratinization. The disorder may feature digital constriction bands (pseudoainhum) which can lead to autoamputation. Surgical excision is the only treatment of pseudoainhum; however, it is fraught with recurrence. We present a 10-year-old boy with pseudoainhum of the right long and small fingers at the distal interphalangeal joints. He underwent 180-degree band excision and z-plasty.

CONCLUSION

Since establishing care 8 years ago, he has undergone treatment of 5 constriction bands with only 1 recurrence. We believe this success can at least be partially attributed to the technique described by Upton.