Patients present with single or multiple inflammatory or sclerotic plaques. These lesions are characterized by circumscribed fibrotic areas involving different levels of the dermis, subcutis, and, sometimes, underlying soft tissue and bone. The linear type can cause problems to underlying tissues such as muscles and bones. Morphea, also known as localized scleroderma, is a disorder characterized by thickening and induration of the skin and subcutaneous tissue due to excessive collagen deposition. However, when too much collagen is made, the skin becomes stiff and hard. Highseverity was defined as presentation with pansclerotic or generalized morphea, losf and subtypes with evidence of high morbidity e. Localized scleroderma causes inflammation in the skin, which triggers connective tissue cells to produce too. Morphea is one of the categories of localized scleroderma 2 3. Systemic involvement in localized sclerodermamorphea anna gorkiewiczpetkow, md, phd, agnieszka kalinskabienias, md, phd. My 25 year old daughter was diagnosed last year with morphea localised scleroderma.
It also features many true stories of patients and caregivers of juvenile scleroderma, morphea, and linear scleroderma. Morphea is an uncommon condition that is thought to affect 2 to 4 in 100,000 people. In these cases, morphea arises first followed by a milder and nonprogressive form of systemic scleroderma. The underlying pathogenesis of morphea is not completely understood at this time, but ultimately results in an imbalance of collagen production and. Mixed forms with different types of localized scleroderma occurring at the same time are observed in about 15% of patients. Morphea is a fibrosing disorder of the skin without systemic involvement. Systemic involvement in localized sclerodermamorphea.
The localized scleroderma ls known as morphea, presents a variety of clinical manifestations that can include systemic involvement. There are two subtypes of this form of scleroderma. Variable elastic fiber changes have been described, but to. Although scleroderma is a spectrum of disorders that can occur at any stage of life, the clinical patterns of childhood scleroderma differ from that of adulthood.
Morphea subtypes are classified according to their clinical presentation and depth of tissue involvement. Up to three in every 100,000 people are diagnosed with localized scleroderma every year, and the disease is more common among. Localized scleroderma is different from systemic scleroderma, in that it only affects the skin, related tissues, and muscles below the tissues. Types of scleroderma there are two main forms of scleroderma. It is an excellent allaround book for patients and caregivers. If you have only a few patches of morphea a type of scleroderma on your skin, the following medicines can be effective. The broad clinical spectrum of ls has led to the development of a number of different. Unlike systemic scleroderma, localized scleroderma usually does not affect internal organs risk factors for localized scleroderma. My impression is that when scleroderma arises in childhood, its more commonly morphea, and when it arises in adulthood it is more commonly systemic, but its not a hard and fast rule, and if doctors make assumptions from this then they can get surprised. Morphea is diagnosed based on findings of skin examination and skin biopsy.
The common plaque type usually causes no problems apart from sometimes an unsightly appearance and tends to fade in time. Learn more about the clinical features, pathophysiology, laboratory diagnosis, treatment, and the prognosis. Localized scleroderma an overview sciencedirect topics. Localized scleroderma morphea is a cutaneous limited fibrosis and represents a wide variety of clinical entities. Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. Localized scleroderma is an inflammatory condition that causes hard and thickened patches of skin to form on different areas of the body. Some patients with generalized morphea also have a band of thickening on an arm or leg as seen in linear scleroderma, another type of localized scleroderma see below. Systemic scleroderma may affect multiple organ systems and is usually classified under autoimmune diseases. With circumscribed morphea another name for discolored patches of skin, you may have a single oval patch or you may see a few patches of morphea.
Morphea is not the same as scleroderma systemic sclerosis, and it. Patients with morphea do not have raynaud phenomenon fingers turning red, white, and blue with cold exposure, redness and swelling of both hands red puffy hands, changes in the blood vessels along the nail edge nailfold capillary changes, or tightening of the skin of the fingers sclerodactyly. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. The prevalence rates of diseases that share scleroderma as a clinical feature are reported ranging from 4 to 253 cases per 1 million individuals. Morphea localized scleroderma is a rare fibrosing disorder of the skin and underlying tissues characterized by skin thickening and hardening due to increased collagen deposition. In children, the linear subtype predominates while in adults plaque and generalized subtypes are most common. Outcome measures and treatment nicolefett,md,andvictoriap. Elastic fiber pattern in sclerodermamorphea request pdf. Localized scleroderma is a nonsystemic skin disease that is seen primarily in children. The treatment of this disease involves the use of longterm immunomodulators. Mri findings in deep and generalized morphea localized.
Localized scleroderma skin lesions can get better or even go away. Scleroderma like skin changes o uctd o eosinophilic fasciitis with eosinophilia o eosinophilia myalgia syndrome o overlap syndromes scleroderma, with sle, or ss, or dmpm, or ra. Treatment of morphea localized scleroderma in adults. Localized scleroderma is characterized by thickening of the skin from excessive collagen deposits. Localized fibrosing disorders include a spectrum of rare conditions that frequently begin in childhood. In a survey conducted in england and ireland, the annual incidence of juvenile ls was 3. Morphea is a form of scleroderma that is more common in women than men, in a ratio 3. Morphea localized scleroderma symptoms and prognosis.
Scleroderma means hard skin and is pronounced skleroderma. The inflammation can trigger connective tissue cells to produce too much collagen, a fibrous protein that is a major part of many tissues. Scleroderma is a disease that causes fibrosis hardening of the skin and sometimes the internal organs. Collagen is a protein normally present in our skin that provides structural support. Morphea localized scleroderma symptoms and prognosis see online here morphea or localized scleroderma is a rare disorder with characteristic clinical features. Disease activity typically persists for three to six years, although some patients develop more persistent or recurring episodes of activity. Nandini moorthy, who wrote the medical overview of juvenile scleroderma. Localized types of scleroderma are those limited to the skin and related tissues and, in some cases, the muscle below. This involves hard, ovalshaped patches on the skin. German guidelines for the diagnosis and therapy of. A rare association of localized scleroderma type morphea.
Localized scleroderma hard skin is an autoimmune condition that causes hardening of the. Localised scleroderma causes one or more patches of hard skin. The treatment of this disease involves the use of long term immunomodulators. Localized scleroderma morphea is a rare disease described in humans, dogs, cats, and a horse. The predominant form of childhood scleroderma is localized scleroderma lsc, also sometimes called morphea, which principally involves the skin, fascia, muscle, and bone. Departmentofdermatology andimmunodermatology,medicaluniversity ofwarsaw,koszykowa 82astr,02 008warsaw,poland abstract localized scleroderma losc, also known as morphea, is a rare fibrosing disorder of the skin. Localized scleroderma is an autoimmune disease in which the immune system causes inflammation in the skin. Localized scleroderma morphea like is a rare disease in dogs and extremely rare in cats and it is an uncommon chronic connective tissue disease. The disease can be either localized to the skin or involve other organs in addition to the skin. Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs. Morphea occurs in childhood as well as in adult life. Morphea scleroderma usually begins as patches of yellowish or ivorycolored rigid, dry skin.
Morphea is a disease of skin the underlying connective tissue. Localized scleroderma morphea and cicatricial alopecia. Based on clinical morphologic findings, morphea can be classified into five groups. The incidence of ls is reported to be approximately 27 cases1,000,000 1, 2. It causes aesthetic impairment due to the sclerotic skin and disability of varying degrees, particularly in the. Adequate studies on the incidence and prevalence have not been performed. Morphea, also known as localized scleroderma, is an idiopathic inflammatory disorder that causes sclerotic changes in the skin.
Morphea or localized scleroderma is a rare disorder with characteristic clinical features. Morphea occurs more frequently in women, especially in adults where female predominance is marked 5. Morphea, also called localized scleroderma, is a sclerosing skin disorder that resembles scleroderma systemic sclerosis in terms of cutaneous histopathological features, but differs demographically and clinically. The patches vary in size and typically have a red border and a thickened paleyellow center. As opposed to generalized morphea, deep morphea is confined to a single or a few mri findings in deep and generalized morphea localized scleroderma marius horger1 gerhard fierlbeck2 jasmin kuemmerledeschner3 nikolay tzaribachev3 manfred wehrmann4 claus d. Claussen1 jan fritz1,5 horger m, fierlbeck g, kuemmerledeschner j, et al. The problem became noticeable when the skin down her left leg looked paler, but also the leg was stiffening and painful and still is giving her pain, at that time she was misdiagnosed with vitiligo due. Symptoms may include areas of thickened skin, stiffness, feeling tired, and poor blood flow to the fingers or toes with cold exposure.
In this article, we will learn about the clinical features including the di. Scleroderma can range from a form localized to the skin to a severe disease the effects the internal organs known as systemic scleroderma. Werth,md philadelphia, pennsylvania morphea is a rare. Several variants have been described, as discussed below. Voices of scleroderma volume 1 features juvenile scleroderma expert dr.