Morphea, also known as localized scleroderma, is a rare fibrosing disorder characterized by round or oval irregular and linear shaped plaques. Histopathology demonstrates numerous lymphocytes and histiocytes located in the dermis scattered amongst collagen bundles. As the disease progresses, the inflammatory cells are replaced with hyalinized connective tissue. Sebaceous glands and hair follicles are absent, but sweat glands are present in reduced numbers amongst a bed of collagen [1,4].

The most widely used classification system is through Mayo Clinic. Morphea is divided into five separate groups namely plaque, generalized, bullous, linear, or deep Morphea. The most frequently encountered group is the plaque Morphea. This form is characterized by round or oval shaped lesions that appear hard and shiny. They are mainly present on the trunk or proximal extremities. Bullous Morphea is a rare form and is characterized by bullous lesions surrounded by plaque Morphea. Deep Morphea is typically seen as a single lesion present around the spine. The skin will appear normal, atrophic, or hardened. Patients are usually asymptomatic with no visceral involvement [3].

Generalized morphea is mostly seen in women and affects two or more body sites. They appear inflamed, pigmented, ill-defined, thickened, and may adhere to the fascia or muscle. Flexion contractures of the joints with muscle-joint symptoms are common. Generalized Morphea does not cause Raynaud’s phenomenon as seen with systemic sclerosis. Linear Morphea is commonly seen in children and appears as a linear streak that may involve the dermis, subcutaneous tissue, muscle, and underlying bone. Approximately 40-70% of children who had Morphea was present in the linear form. Lesions may be present in the scalp, extremities, or face. If the bone is involved, this may interfere with normal growth and development of the child. If the scalp is the involved area it may lead to areas of Alopecia.

Approximately 20% of patients with localized scleroderma will develop extra cutaneous manifestations such as arthritis, seizures, or uveitis. Neurological symptoms are the most encountered systemic manifestation presenting as complex partial seizures. Brain changes were identified in approximately 75% of the cases who were evaluated with an MRI. Children may develop limb length discrepancy and joint contractures requiring orthopedic evaluation. Linear scleroderma has an increased prevalence of autoimmune diseases such as diabetes, Hashimoto’s thyroiditis, Grave’s disease, and ulcerative colitis.

Treatment for Morphea may include topical creme, phototherapy or systemic therapy. Moderate to high potency topical corticosteroids are recommended as first line topical treatment for superficial circumscribed Morphea. Tacrolimus 0.1% may also be used for superficial Morphea if there is no improvement with topical corticosteroids or simultaneously. Topical calcipotriol 0.005% crème may also be used in combination with a topical corticosteroid crème.

Phototherapy with UVA1 has demonstrated excellent short-term relief. The recurrence rate at two years was 44.5% and 48.4% at three years. Phototherapy should primarily be used for superficial Morphea. Systemic corticosteroids such as prednisone may be used as treatment, but the recurrence rate is 45% after treatment is discontinued. Long-term use of systemic corticosteroids also led to unfavorable side effects. Methotrexate, with or without systemic corticosteroids is also an option for treatment with an 80-94% response rate.  Mycophenolate Mofetil may also be used an alternative treatment to Methotrexate [2].