Paediatric Dupuytren's disease

Abstract

Dear Editor, This paper presents the first documented case of paediatric Dupuytren's disease in Australasia. An 8-year-old boy was referred by his general practitioner with a 6-month history of nodular mass in the right palm. There was no history of trauma. On examination, a 7-mm diameter mass was located in the right mid-palm between the palmar creases opposite the third web space. The mass was adherent to the overlying skin; however, the finger range of motion was preserved. Ultrasound reported a semi-cystic, semi-solid mass in the subcutaneous tissue. Surgical excision was performed to obtain a histological diagnosis. Intraoperatively the nodule was noted to be within the longitudinal cord of palmar fascia and had a macroscopic appearance typical of Dupuytren's disease (Fig. 1). Histology confirmed the diagnosis. The post-operative period was unremarkable and follow-up at 3 months revealed no evidence of recurrence. Intra-operative view of palmar fibromatosis. Only six cases of histologically proven Dupuytren's disease have been reported worldwide in children under 10 years of age, including two infants aged 6 and 10 months.1–3 Fetsch identified a further two cases among a total of 56 cases of paediatric fibromatosis; however, patient age was not disclosed.4 Of the six cases, there was a male-to-female ratio of five to one, with a mean age at presentation of 4.8 years. Most common locations were the palm or little finger and fixed flexion deformities were present at the proximal interphalangeal joint in 60% of cases, with the deformity ranging from 20 to 500. All cases were treated surgically, with five undergoing fasciectomy. To obtain up-to-date follow-up data we contacted clinicians of the above cases and obtained information on five patients. This revealed recurrence in only one patient, with follow-up ranging from 18 months to 5 years. Recurrent Dupuytren's disease was originally reported in a second child; however, this was histologically misdiagnosed.3 A review of the original slides revealed epitheloid sarcoma, highlighting the care required when diagnosing recurrent disease in the paediatric age group. The presence of any atypical clinical or histological features is an indication for performing epitheloid marker assays including cytokeratin 8 and 18 and epithelial membrane antigen.