A 55-year-old caucasian woman, with bilateral sensorineural hearing loss, came to our attention complaining of severe pain in the fifth digit of the right hand. The pain had been mild during the last year and became acute in the last two months. Clinical examination showed “honeycomb” palmar keratosis, starfish keratosis on the dorsal side of both hands and feet (which the patient had been suffering since the age of five), digital constriction bands in the proximal interphalangea (PIP) joint of the right fifth digit and skin dyschromia (). Neurovascular deficit with pain, paresthesia and hypoesthesia of the fifth finger were also present. Her father, sister and grandmother had the same syndromic pattern. Based on these clinical findings a diagnosis of Vohwinkel syndrome was made. Plain radiographs of the right hand showed periosteal thinning of the proximal phalanx of the fifth digit and soft tissue alteration ().\nMR confirmed the periosteal thinning and showed a lack of visualization of a segment (8 mm) of the extensor tendon at the proximal interphalangeal joint of the fifth digit, compatible with a complete lesion (). Marked thinning of the fifth digit’s flexor tendon, thickening of the flexor tendons of the other fingers, and thinning of their extensor counterparts, were also evident.\nConservative surgical treatment was proposed to the patient, but owing to the high recurrence rate described in the literature she refused this type of approach and preferred amputation of the proximal phalanx of the fifth digit of the right hand.\nIn anesthesia, a skin incision was made through the proximal interphalangeal joint and a capsulotomy was performed. We then proceeded with the amputation and disarticulation of the head of the proximal phalanx, followed by proper hemostasis, tendon plasty procedure and suturing (). The incision was medicated with sterile gauzes, and an elastic dressing was applied.\nPost-surgical follow-up consisted in anterior-posterior and latero-lateral radiographs of the right hand, and an inhospital observation period of 24 hours during which neither vascular nor nervous deficits emerged. Optimal articular range of motion was maintained and pain was greatly reduced. Histological examination showed (severe hyperkeratosis, hypergranulosis, and mild acanthosis, of the cutaneous component consistent with the diagnosis of Vohwinkel Syndrome ().\nAt 15 days follow-up, the surgical wound was clean, no sign of infection was evident, and the patient didn’t complain any pain. At 12-month follow-up, the patient did not report pain, and plain radiographs showed no abnormalities at the site of amputation.
A 35-year-old man presented to us with pain and fissuring in his left fifth toe []. The patient noted a groove occurring in the inner aspect of his toe 5 years back, which gradually increased. Similar grooves started appearing in his fourth toe bilaterally since the past 2 years. At the age of 8 years, he had a similar problem affecting the right fifth toe, which gradually led to the loss of the digit as accounted by his father. On examining the father who accompanied him, it was noted that he also had similar but asymptomatic grooving involving the right fourth and fifth toes and left fifth toe []. The grooving was most severe in the fourth toe. The patient presented with hope to control the disease from spreading to all his digits and to prevent any further loss of his digits.\nThe patients were otherwise healthy; all his baseline investigations were done and X-ray of the foot was taken. Antibiotics were given for control of secondary infection and he was advised to prevent trauma to the foot. After control of infection, the patient was referred to plastic surgery for favor of Z plasty procedure. The patient came for regular reviews. On subsequent visits, the patient noted significant reduction in the pain following the procedure with reduction in the progression of the fissuring. The improvement was better for the son than for the father after both underwent Z plasty [Figures and ].