What Is Plaque Morphea?
What Is Plaque Morphea?
Plaque morphea is a superficial type of lesion found in the dermis of the skin. The surface becomes smooth, shiny and hairless with loss of sweat glands and over time the affected area may become sunken and depressed. It can further be divided into several types including, plaque morphea itself that consists of sclerosis of red, swollen, oval to round, white-yellow color along with central depression, in addition to red to purple edges (lilac ring). The second form is the ring morphea, in which circular type of sclerosis is seen affecting the extremity. The third form is the guttate morphea, which consists of several small grouped lesions of several millimeters that are white, shiny, depressed without any sclerotic changes and are mostly found in the chest area. The fourth type is keloid morphea, which is characterized by typical plaques with separate nodules or united nodules having appearance of a keloid scar. The fifth type is lichen sclerosus et atrophicus that consists of white atrophic macules and small plaques. The sixth variety is atrophoderma of Pasini and Pierini, which has a questionable category and presents as hyperpigmented and asymptomatic patches with “cliff drop borders” found mostly on the trunk and extremities.
Other than plaque morphea, the widely accepted classification in medical fraternity classifies morphea (localized scleroderma) into four subgroups. These include-
Bullous Morphea: Bullous morphea consists of hard subepidermal bullae along with typical morphea or deep morphea.
Generalized Morphea: Generalized morphea is a more severe form of morphea that extends in a wide area of the skin and can also involve the underlying muscles.
Linear Morphea: Linear morphea as the name implies is characterized by one or more linear lines that may affect the dermis, subcutaneous tissues, muscle and bone. It can be found on the extremities, scalp, or face of children or adults. En coup de sabre is a deep form of linear morphea that affects scalp and temple region of a person. This may affect the underlying skull bone that may cause shrinkage and loss of hair in the area. When linear morphea crosses a joint, may affect the mobility or functioning of the limb.
Deep Or Pansclerotic Morphea: Deep or pansclerotic morphea involves the underlying subcutaneous tissues, muscles and bones. It is mostly found in children and can be disabling when a bone is involved. It can lead to stunted growth of the involved bone.
The treatment of morphea should be based on the type of morphea as most of them are benign type and regress spontaneously within 3-5 years with a favorable outcome. Plaque morphea is a self-limiting form with better esthetics, so should be treated with topical corticosteroids, calcipotriol (vitamin D substitute), tacrolimus 0.1%, imiquimod 5% or hyaluronidase to reduce inflammation and prevent progression of the lesion. Phototherapy has also proven successful for the treatment of morphea.
Scleroderma is a chronic autoimmune disease targeting connective tissue and blood circulation leading to fibrosis and surrounding blood vessel obliteration. Scleroderma is categorized depending on the extent of skin and organ involvement and is mainly of two types, namely, localized scleroderma (morphea) and systemic scleroderma.
Systemic scleroderma is a more life-threatening variant of scleroderma in which along with diffuse sclerosis of skin and underlying connective tissue, calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia, internal organs (lungs, kidneys, gastrointestinal tract) are also affected.
Localized scleroderma or morphea is a benign and more common form of scleroderma that only affects skin, subcutaneous or underlying muscles of trunk and extremities. Morphea is usually chronic, self-limiting with a favorable prognosis. Both localized and systemic sclerodermas are more commonly seen in women than in men. The time of occurrence is mostly during childhood, adolescent or young adult phase, but people of any age may be affected.
Excess collagen deposition by fibroblasts is the main culprit behind morphea; however, the exact cause behind this aberrant behavior of fibroblasts is still unclear.
Autoimmunity, genetics, infection, trauma or toxicity have been suggested etiology and trigger factors.