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 54-year-old female from the Northern region of Cameroon, presented on January 12, 2018 to our primary health care hospital with a 6-month history of a painful circular constriction band at the base of her right fifth toe. There was no history of trauma, ligation or bangles on the toe nor laceration of the toe. Her past history was not notable for any chronic disease such as diabetes, ischaemic heart disease, peripheral vascular disease, skin pathologies, HIV/AIDS and psychiatric disorder. Also, she was a non-smoker. There was no family history of similar skin disease.\nOn examination, she was conscious, oriented, and was not ill-looking. Her blood pressure was 138/74 mmHg, pulse rate 102 beats/minute and regular, respiratory rate 18 breaths/minutes and temperature 36.4 °C. All distal pulses were present and of normal volume. There was no regional lymphadenopathy. On examination of the right foot, the fifth had a circular constrictive band at the base of the proximal inter-phalangeal joint (see Fig. ). A provisional diagnosis of a toe infection was made. She was admitted and administered cefuroxime 1.5 g/12 h intravenously (IV), metronidazole 500 mg/8 h IV, paracetamol 1 g/6 h IV, diclofenac 75 mg/ 12 intramuscularly and wound dressing every 48 h. Her clinical re-assessment 1 week later was notable for progression of the constricting band which made contact with the bone until a stage of near auto-amputation of its distal segment (see Fig. ). The distal aspect of the toe was bulbous and oedematous. The affected toe was very mobile and any attempt to actively or passively mobilize it caused excruciating pain. There was bilateral plantar hyperkeratosis. The rest of her clinical assessment was normal. X-ray of the right foot could not be done because it was not available in our health facility. A laboratory panel requested on her admission revealed complete blood count (CBC): white blood cell count 8,400/mm3, haemoglobin 12.1 g/dl, platelet count of 348,000/mm3; raised C-reactive proteins at 48 mg/l and an erythrocyte sedimentation rate at 46 mm 1st hour and 89 mm 2nd hour. The worsening of her toe’s pathology prompted a quick diagnostic review, thereby advocating for the diagnosis of stage four ainhum with impending auto-amputation based on the history and clinical findings. After proper preoperative workups consisting of a complete blood count, prothrombin time and activated thromboplastin time (which were all normal), the constricting band was excised under local anaesthesia by infiltration of lidocaine 2% at its base, the right fifth toe was disarticulated at right fifth metatarsophalangeal joint and the skin was closed surgically. Histopathological examination of the amputated toe was not accessible. Her postoperative course was uneventful and she was discharged on day seven after wound healing. Follow-up to 1 year was equally uneventful.