Patients with morphea are not at increased risk of developing systemic sclerosis. It is a form of scleroderma patches most often occur on the abdomen, stomach, and back, and sometimes on the face, arms and legs. Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma morphoea in children. The main subtypes are plaque morphea, linear scleroderma, generalized morphea, and pansclerotic morphea. Scleroderma is a chronic connective tissue disease characterized by hardening of the skin and is divided into two major groups. In this article, we will learn about the clinical features including the di. Treatment of localized scleroderma depends on the clinical subtype.
The disease can be either localized to the skin or involve other organs in addition to the skin. These then become hard, slightly depressed, ovalshaped plaques which usually have a whitish or yellowish center surrounded by a pinkish or purplish halo. Localized scleroderma is a condition characterized by varying degrees of hardening of the skin, which happens because of abnormal collagen deposits unlike systemic scleroderma, localized scleroderma rarely involves internal organs. Based on clinical morphologic findings, morphea can be classified into five groups. Patients present with single or multiple inflammatory or sclerotic plaques. Morphea genetic and rare diseases information center. Immunopathogenesis of pediatric localized scleroderma. Morphea is a fibrosing disorder of the skin without systemic involvement. Localized scleroderma can occur in several different forms, including linear scleroderma where the lesion appears as a line or streak and circumscribed morphea where the lesion appears as a roundish lesion. Localized scleroderma, known as morphea, may occur in patches or linear lesions, or it may be superficial. Good response of linear scleroderma in a child to ciclosporin. Hi cristina, yes i understood that you have been diagnosed with localized scleroderma. Localized scleroderma ls is a complex disease characterized by a mixture of inflammation and fibrosis of the skin that, especially in the pediatric population, also affects extracutaneous tissues ranging from muscle to the central nervous system.
In children, the linear subtype predominates while in adults plaque and generalized subtypes are most common. 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. Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. This disorder results when there is excessive collagen deposition in the deeper part of the skin called the dermis and the fatty subcutaneous tissues of the skin. Treatment of linear scleroderma with oral 1,dihydroxyvitamin d3 calcitriol in seven children. Unlike systemic scleroderma, localized scleroderma usually does not affect internal organs risk factors for localized scleroderma. Morphea localized scleroderma symptoms and prognosis see online here morphea or localized scleroderma is a rare disorder with characteristic clinical features. When the morphea goes deeper, uva1 phototherapy may be prescribed. However, systemic scleroderma is a clinical diagnosis based on symptoms.
According to the standard nomenclature of diseases and operations,1 it is classified into generalized, progressive, and localized morphea. Morphoea american spelling, morphea is characterised by an area of inflammation and fibrosis thickening and hardening of the skin due to increased collagen deposition. The two main types are systemic scleroderma and localized scleroderma. Localized scleroderma is an inflammatory condition that causes hard and thickened patches of skin to form on different areas of the body.
Localized scleroderma is characterized by thickening of the skin from excessive collagen deposits. Morphea or localized scleroderma is a rare disorder with characteristic clinical features. The patches appear on any area of the skin, initially as pinkish red rounded or oval spots varying in size, with subsequent thickening in the center of the focus. There are two generally recognized types of localized scleroderma. Morphea is not the same as scleroderma systemic sclerosis, and it. Update on the classification and treatment of localized scleroderma. Pulsed highdose corticosteroids combined with lowdose methotrexate in severe localized scleroderma. Mri findings in deep and generalized morphea localized. The localized scleroderma ls known as morphea, presents a variety of clinical manifestations that can include systemic involvement. The treatment of this disease involves the use of long term immunomodulators. An association has been found with a parasitic disease toxoplasma. But it can affect tissue directly under the skin such as the muscles, nerves, and bones.
It is very unusual for localized scleroderma to develop into the systemic form. Morphea subtypes are classified according to their clinical presentation and depth of tissue involvement. Localized scleroderma can be divided into five general subtypes. Morphea is a frequently mild, benign, and selflimiting skin disease with a less than 1% reported chance of progressing to systemic scleroderma. Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs. Histopathological changes in morphea and their clinical.
There are two subtypes of this form of scleroderma. Morphea is classified according to the localization of the lesions and the depth of tissue involvement into localized or circumscribed limited to one or. Localized linear scleroderma is a type of localized scleroderma of unknown cause. Disease activity typically persists for three to six years, although some patients develop more persistent or recurring episodes of activity. The localized scleroderma cutaneous assessment tool is a promising. The treatment of this disease involves the use of longterm immunomodulators. Uva1 produces long wavelengths that can deeply penetrate the skin to reduce hardness. 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.
Localized scleroderma designates a heterogeneous group of sclerotic skin disorders. Morphea is diagnosed based on findings of skin examination and skin biopsy. Morphea morfeeah comes from a greek word that means form or structure. Collagen is a protein normally present in our skin that provides structural support. This is a pdf file of an unedited manuscript that has. Localized scleroderma is different from systemic scleroderma, in that it only affects the skin, related tissues, and muscles below the tissues. A morphea this is the most common type of localized scleroderma. Morphea scleroderma usually begins as patches of yellowish or ivorycolored rigid, dry skin. Genetic architecture of linear localized scleroderma zurich open.
Localized types of scleroderma are those limited to the skin and related tissues and, in some cases, the muscle below. Morphea occurs more frequently in women, especially in adults where female predominance is marked 5. Morphea is distinct from systemic sclerosis scleroderma, an autoimmune connective tissue disorder characterized by acral or diffuse cutaneous sclerosis and frequent systemic manifestations. Treatments help to control itch and decrease the growth and formation. Morphea is a sufficient and less terrifying name for these disorders than localized scleroderma. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. Classification of morphea localized scleroderma mayo clinic. Systemic involvement in localized sclerodermamorphea. Scleroderma is a word used to describe a spectrum of conditions characterized by hardening andor thickening of the skin and fibrosis of the tissues involved. Localized scleroderma is more common in children, who have a condition. A type of light treatment called narrowband uvb treatment can be helpful. In this disorder, a hardened line of skin can occur in any part of the body.
May i please tell you that there are rules on this site against solicitation of sales, and that there is currently no known cure for scleroderma, and that some herbs can hurt scleroderma patients more than they help. A rare association of localized scleroderma type morphoea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis, case report. Abstract morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. Morphea is a skin condition that causes patches of reddish skin that thicken into firm, ovalshaped areas. It is important to understand that localized scleroderma is different from the form of scleroderma which affects internal organs, called systemic sclerosis or, often incorrectly stated, as systemic scleroderma. Besides the skin hardening, there can be changes in skin color and texture, and the underlying tissues may fail to grow normally. Localized fibrosing disorders include a spectrum of rare conditions that frequently begin in childhood. Generalized morphea is a subtype of localized scleroderma, which lacks systemic manifestations and displays widespread, multiple, wellcircumscribed, indurated. Linear scleroderma and morphea can coexist in the same patient. Update on morphea journal of the american academy of. Evidencebased treatment options of morphea are limited secondary to the rarity. Histopathological features in morphea localized scleroderma and their clinical correlates are poorly described. Morphea localized scleroderma symptoms and prognosis.
This skin disorder if often classified as generalized. The term scleroderma covers various types of morphoea and systemic sclerosis subtypes of morphoea vary according to the location of involved skin. Generalized morphea is a rare subtype of localized scleroderma and displays widespread. I was just saying that it is possible to be ana positive without having systemic scleroderma. While this does not imply that patients with localized scleroderma evolve to develop these. It can be clinically classified in different forms. These lesions are characterized by circumscribed fibrotic areas involving different levels of the dermis, subcutis, and, sometimes, underlying soft tissue and bone. Morphea is the preferred term in the clinical setting to avoid patient confusion with systemic sclerosis ssc which could cause unnecessary confusion and concern. Morphea, also known as localized scleroderma, is an idiopathic inflammatory disorder that causes sclerotic changes in the skin. Symptoms may include areas of thickened skin, stiffness, feeling tired, and poor blood flow to the fingers or toes with cold exposure. In children, linear morphea on the arms and legs can affect the underlying bone and interfere with bone growth.
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. Scleroderma is a disease of unknown cause and many synonyms that varies from a mild localized form to a severe generalized type with systemic manifestations. Although developmental origins have been hypothesized, evidence points to ls as a systemic autoimmune disorder, as there is. Learn more about the clinical features, pathophysiology, laboratory diagnosis, treatment, and the prognosis. Treatment of morphea localized scleroderma in adults. Morphea, in fact, is only one particular type of localized scleroderma. Localized scleroderma morphea is a cutaneous limited fibrosis and represents a wide variety of clinical entities. Localized scleroderma is a skin condition that can take. This entity is subdivided into linear scleroderma, plaque. The reader will be able to apply these findings to patient management.
If morphea crosses a joint, the thickening may limit joint movement. Systematically describe histologic changes of morphea in a large, well annotated cohort and determine the association between histopathology and clinical features. German guidelines for the diagnosis and therapy of localized. The skin becomes waxy yellow and smooth, and loss of hair occurs.
Morphea, also known as localized scleroderma, is an idiopathic, inflammatory disorder that causes sclerotic changes in the skin. A rare association of localized scleroderma type morphea. Morphea also may be underreported, as physicians may be unaware of this disorder, and smaller morphea plaques may be less often referred to a dermatologist or rheumatologist. Localized scleroderma ls typically presents in childhood with an estimated annual age and sex adjusted incidence rate of per 100,000 children and prevalence of 2 per 1,000 children. 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.
Morphea is characterized by skin thickening with increased quantities of collagen in the indurative lesion. Scleroderma was divided into morphea guttate type, linear scleroderma, generalized morphea, hemiatrophy, and systemic scleroderma. Alife table analysis of clinical an demographic forrns in the derrnallesions of morphea and lichen in male v. Localized scleroderma is a distinct entity, differing widely from the systemic form by almost exclusive cutaneousinvolvement. Morphea localized scleroderma for professionals morphea is a disorder which is characterized by thickened skin lesions. For some people, localized scleroderma is serious and disabling. The first signs of the disease are reddish patches of skin that. However, when too much collagen is made, the skin becomes stiff and hard.
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