Diagnosis and management of morphoea in children: an overview

Area of Science:

• Dermatology
• Paediatric Rheumatology
• Immunology

Background:

• Paediatric morphoea (localized scleroderma) is an inflammatory, sclerosing skin disorder.
• Often considered benign, it can be chronic, relapsing, and lead to significant morbidity, especially in early childhood.
• Early recognition is challenging, with key signs including band-like distribution, tissue atrophy, sclerosis, and hair loss.

Purpose of the Study:

• To review the clinical presentation, long-term outcomes, and treatment of paediatric morphoea.
• To highlight the potential for extracutaneous complications and the need for effective management strategies.

Main Methods:

• Literature review of paediatric morphoea, focusing on clinical features, disease course, and therapeutic interventions.
• Analysis of current first-line and second-line treatment options, including corticosteroids, methotrexate, mycophenolate mofetil, and abatacept.
• Discussion of adjunctive therapies such as autologous fat injections.

Main Results:

• Paediatric morphoea can lead to significant morbidity due to chronicity, relapses, and extracutaneous manifestations (up to 20%), including arthritis and neurological symptoms.
• Corticosteroids and methotrexate are effective first-line treatments, partially reversing skin changes but not fully preventing atrophy.
• Relapse rates range from 25%-48% after treatment cessation, necessitating long-term follow-up.

Conclusions:

• Paediatric morphoea is a complex condition requiring vigilant management beyond its perceived benign nature.
• Effective first-line therapies exist, but second-line options like mycophenolate mofetil and abatacept, along with autologous fat injections, offer further therapeutic avenues.
• Long-term follow-up is essential due to the relapsing nature and potential for serious complications.