From the Guidelines
Linear morphea is a subtype of juvenile localized scleroderma characterized by one or more linear streaks or bands of hardened, discolored skin, typically affecting the limbs, face, or scalp, and can extend deep into underlying tissues, potentially causing muscle atrophy and joint contractures if left untreated. According to the most recent and highest quality study 1, linear morphea is a rare condition in children, with an incidence of 3.4 cases per million children per year, and a female to male ratio of 2.4:1, with a mean age at onset of approximately 7.3 years. The severity of the disease varies widely, and patients with suspected linear morphea should be referred to a specialized pediatric rheumatology center for clinical assessment and treatment.
Key Characteristics of Linear Morphea
- Typically affects the limbs, face, or scalp
- Can extend deep into underlying tissues, potentially causing muscle atrophy and joint contractures if left untreated
- Begins as reddish or purplish patches that gradually become thickened, waxy, and ivory-colored with a violet border
- Most common in children and young adults, though it can affect people of any age
Treatment Options
- Topical corticosteroids like clobetasol 0.05% applied twice daily for mild cases
- Systemic medications such as methotrexate (15-25 mg weekly) combined with oral prednisone (1 mg/kg/day, gradually tapered) for more severe cases
- Phototherapy with UVA1 or PUVA can also be effective, as recommended by the consensus-based guidelines 1
- Methotrexate should be maintained for at least 12 months before tapering, as recommended by the expert panel 1
Importance of Early Treatment
While linear morphea is not life-threatening, early treatment is crucial to prevent permanent tissue damage and functional limitations. The condition results from an autoimmune process where the body's immune system inappropriately attacks connective tissue, leading to excessive collagen production and fibrosis. Therefore, it is essential to initiate treatment promptly to prevent long-term consequences.
From the Research
Definition and Characteristics of Linear Morphea
- Linear morphea, also known as linear scleroderma, is a localized form of scleroderma characterized by the presence of lesions that follow a linear distribution pattern 2.
- It is a chronic autoimmune disorder that can have a significant impact on the patient's daily life due to the unaesthetic evolution of the cutaneous lesions 3.
- Linear morphea can affect underlying structures such as muscles and bones, leading to functional limitations 2.
Clinical Presentation and Forms
- Linear morphea en coupe de sabre (LM) usually arises in childhood, but it can also occur in adulthood, showing a more aggressive course with an increased risk of systemic involvement 3.
- Linear morphea can exhibit lesions that follow the lines of Blaschko, mimicking lichen striatus 2.
- The disease can be divided into different subtypes, including morphea, linear scleroderma, and generalized morphea 4.
Treatment Options
- Methotrexate is the first-line treatment for linear morphea, although systemic steroids, topical agents, hyaluronic acid injections, and hydroxychloroquine or mycophenolate mofetil are also valid therapeutic options 3.
- Platelet-rich plasma (PRP) injection can be considered a valid and safe alternative treatment for linear morphea 3.
- Baricitinib has been shown to be effective in treating linear morphea, especially in cases that follow the lines of Blaschko 2.
- Treatment should be decided according to the severity and extent of lesions, and may include phototherapy, methotrexate, systemic corticosteroids, calcipotriene, and topical tacrolimus 5, 6.