Short- and long-term outcome of linear morphoea in children

Abstract

Background: Linear morphoea (LM) is a rare fibrosing disorder of the limbs or the face that may cause functional disability and severe aesthetic sequelae. Despite a wide range of therapeutics reported for LM, there is currently a lack of consensus on the optimal therapy. Little is known about the long-term outcome of this disease.

Objectives: To describe the short- and long-term outcome of a large series of patients with LM acquired in childhood.

Methods: A retrospective chart review of 52 paediatric patients with LM seen in our centre during a 20-year span (1990-2010) and a telephone survey in 2011 to assess the long-term outcome of these patients.

Results: Limbs were affected twice as often as the face, with a higher proportion of female patients. Stabilization was obtained after a mean disease duration of 5·4 years. Patients sometimes experienced long stretches of disease quiescence followed by reactivation; 31% of patients reported active disease after 10 years. All but one patient had aesthetic sequelae, and 38% had functional limitations. The effectiveness of methotrexate and systemic corticosteroids was apparent in the short term.

Conclusions: LM needs prolonged monitoring as the disease can have very long periods of quiescence followed by reactivation. The combination of methotrexate and systemic corticosteroids was effective in the early stages of the disease but did not seem to prevent long-standing active disease or relapse in the long term.