Morphea

Management Strategy

Initial evaluation of a patient with morphea begins with a history and physical examination to determine morphea subtype, lesion activity level, depth of involvement, rate of progression, and presence of extracutaneous manifestations. The following elements of the history are of particular importance. Establishment of a clear timeline of lesion onset and progression aid in determination of activity level. Careful assessment of additional findings such as Raynaud phenomenon or acrosclerosis may distinguish morphea from systemic sclerosis (SSc). Importantly, morphea may occur concurrently with arthritis, fasciitis, and myositis, typically in the context of soft tissue lesions of linear or generalized subtype. Neurological manifestations and ocular complaints occur most often with linear head and neck lesions. Additionally, patients with linear head and neck lesions should be questioned regarding jaw or oropharynx changes. Patients with generalized morphea should be questioned regarding genital/perianal changes.

The aim of the physical exam is to confirm the diagnosis, assess disease activity and extent (body surface area and depth of involvement), and assign subtype. Lesion activity and damage can be determined by the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT), a validated clinical measure for evaluating patients with morphea. Active morphea is defined by erythema, peripheral induration, and presence of new or enlarging lesions, quantified by the LoSCAT activity component. Erythema and induration are obvious if the superficial levels of the skin and soft tissue are involved. However, deep morphea lesions involving the subcutis are difficult to assess. Palpation of the lesion may help determine depth and extent of sclerotic plaques, but MRI or ultrasound is often needed.

Inactive morphea, the sequalae of active lesions, can be quantified by the damage component of the LoSCAT and is defined by peripheral dyspigmentation, dermal or soft tissue atrophy, telangiectasias, and central sclerosis causing a yellow-white color. Lesion sclerosis is eventually replaced by dermal/subcutaneous atrophy with shiny skin, prominent vessels, and cliff-drop atrophy, or in the case of deep lesions loss of normal body contour. Hair regrowth may occur as lesions become inactive.

Importantly, only active lesions (as seen in the photograph) respond to immunosuppressives or phototherapy. Therefore, patients who exclusively have inactive lesions should receive supportive care and monitoring for reactivation. Determination of activity and damage guide treatment modalities.

SSc and morphea are distinct entities distinguishable by physical exam. Features of SSc including nailfold capillary changes, sclerodactyly, acrosclerosis, Raynaud phenomenon, and salt and pepper skin pigmentation changes are absent in morphea. Morphea patients lack the hallmark antibodies of SSc, including RNA polymerase, topoisomerase, and centromere antibodies. Presence of these physical exam findings and antibodies is concerning for SSc.