Morphea is an inflammatory skin disorder that is known as morphea scleroderma or localized scleroderma. Scleroderma means “hard skin” and is of two types’ systemic scleroderma and localized scleroderma. Localized scleroderma or morphea affects only the skin, whereas, systemic scleroderma affects the internal organs as well. in addition to involvement of internal organs in systemic scleroderma, there is also presence of Raynaud’s phenomenon (reduced blood flow to the toes or fingers due to cold or stress), telangiectasias (spider veins), sclerodactyly (claw like fingers or toes due to tightening and hardening of their skin), along with changes in the nailfold capillary.
Morphea is caused due to excessive collagen production that leads to thickening and hardening of the skin. It may be subdivided into various types, namely, linear, circumscribed, and generalized and pansclerotic depending on the extent and depth of skin tissue involvement.
Can Morphea Be Cured?
Morphea is a self-limiting condition, in which the lesions usually regress spontaneously in 3-5 years, without the need of any treatment; however, treatment is given to limit the progression of symptoms. The earlier the initiation of treatment, the better is the prognosis. Although, there is no permanent cure for this ailment, its symptoms can still be managed from spreading further and limiting morbidity.
Active lesions, which have less than 3 months of time of initiation, are best responsive to treatment. Circumscribed morphea may be best managed with skin creams and ointments or targeted phototherapy to the lesions. Topical or intralesional corticosteroids are helpful in reducing the inflammation and progression of the lesion. Tacrolimus 0.1% ointment, calcipotriene and imiquimod 5% cream have shown some promising results in curbing the disease.
More aggressive therapy is required for generalized, linear and deep morphea as these can be potentially disabling conditions. Systemic corticosteroids (IV methyprednisolone or oral prednisone) in addition to methotrexate have been used successfully for severe and progressive morphea. Methotrexate can be used alone for the treatment of generalized form of morphea and in patients resistant to it, mycophenolate mofetil is found effective.
Phototherapy is also found beneficial as an adjunct to systemic corticosteroids and methotrexate treatment in severe or refractory cases owing to its reduced side effects when compared to immunosuppressive agents. Low, medium and high doses of UVA have been used and all of them have been found to be useful; however, low doses of UVA1 and UVB have been less efficacious than medium or high doses of UVA1 due to limited penetrability in the dermis. Phototherapy is best used in combination therapy along with corticosteroids and calcipotriene.
In some severe cases of linear or deep morphea, orthopedic surgical intervention might be needed for joint deformities to release joint contractures or for limb-lengthening procedures. Plastic surgery may be employed to correct deformities of the face or scalp region where esthetics are of prime importance.
Causes Of Morphea
The cause of morphea is still unknown, but it has been linked to autoimmune response of the body to the healthy cells and tissues and increased prevalence has been seen in personal and family history of the patient. There have been various triggers for morphea, which include radiation therapy related to breast cancer or other malignancies (can be found near of away from the site of irradiation and even after years of previous irradiation), infections including borreliosis, hepatitis B, Epstein-Barr virus, measles and varicella. Repeated trauma, medication induced, vaccinations, hormones and chemical exposure have been some other triggers.
Morphea is mostly an asymptomatic condition, but on rare occasions it might be painful or itchy. It consists of red to purple plaques of the skin with surrounding swelling. The center may turn white in time, which later on becomes a hyperpigmented patch upon resolution. Deep variety of morphea may also involve the underlying tissue and muscles. These are mostly found in the trunk region, followed by extremities and face area. Muscle weakness or limb limitation may be a part of deep varieties of morphea in which there is involvement of underlying muscles or bones.