The disorder can be mild, moderate or severe, depending on how much skin and connective tissue is affected. Localized types of scleroderma are those limited to the skin and related tissues and, in some cases, the. 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 to the skin colour tone changing. Localized scleroderma is a skin condition that can take many. Morphea is characterized by patches of yellowish or ivorycolored rigid, dry skin that become hard, slightly depressed oval plaques.
This involves hard, ovalshaped patches on the skin. Systemic scleroderma occurs throughout the body, affecting internal organs. Localized scleroderma an overview sciencedirect topics. The topic localized scleroderma morphea you are seeking is a synonym, or alternative name, or is closely related to the medical condition morphea. 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. Abstract morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. Classification of morphea localized scleroderma sciencedirect. Departmentofdermatology andimmunodermatology,medicaluniversity ofwarsaw,koszykowa 82astr,02 008warsaw,poland abstract localized scleroderma losc, also known as morphea, is a rare fibrosing disorder of. Apr 03, 2012 localized scleroderma is characterized by thickening of the skin from excessive collagen deposits. Evidencebased recommendations for treatment fett nm.
Localized scleroderma is a rare disease of unknown etiology. Localized scleroderma can be divided into five general subtypes. An association has been found with a parasitic disease toxoplasma. Localized scleroderma localized scleroderma is almost always a purely skin condition, and is virtually never associated with the severe and potentially life threatening complications of ssc. Morphea is a localized type of scleroderma that is characterized by thickened skin patches, which can increase and decrease in size. Morphea localized scleroderma symptoms and prognosis. Scleroderma is a chronic condition that affects the connective tissue. When the morphea goes deeper, uva1 phototherapy may be prescribed. Morphea morphea consists of patches of thickened skin that can vary from half an inch to six inches or more in diameter. Scleroderma nord national organization for rare disorders.
Although a wide range of therapeutic strategies have been reported in the literature, a lack of agreement on treatment specifics and accepted methods for clinical assessment of have made it difficult to compare approaches and identify optimal therapy. Morphea usually occurs on the trunk, although it may be widespread generalized morphea. It is a group of diseases that cause abnormal growth of connective tissue. Based on clinical morphologic findings, morphea can be classified into five groups. Ssc, when advanced, is often compromised with severe gastroesophageal reflux disease gerd, which may be lethal in a worstcase scenario. Morphea is distinct from systemic sclerosis scleroderma, an autoimmune connective tissue disorder characterized by acral or diffuse cutaneous sclerosis and frequent systemic manifestations. Keloidal scleroderma presents as multiple keloidlike lesions that occur in the absence of preceding trauma or injury and can be associated with localized or systemic.
The localized scleroderma ls known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Morphea is diagnosed based on findings of skin examination and skin biopsy. Lesions are usually limited and most commonly just one lesion is found. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. In this article, we will learn about the clinical features including the di. Adequate studies on the incidence and prevalence have not been performed. The common plaque type usually causes no problems apart from sometimes an unsightly appearance and tends to fade in time.
Morphea is a fibrosing disorder of the skin without systemic involvement. Guido bonoldi head of medicine ii, busto arsizio hospital over the last thirty years or so, in the face of the rapidly developing scientific and technological progress that characterizes modern medicine, a progressive process of the specialization of medical skills has become the norm. A rare association of localized scleroderma type morphea. These lesions are characterized by circumscribed fibrotic areas involving different levels of the dermis, subcutis, and, sometimes, underlying soft tissue and bone. Morphea is not the same as scleroderma systemic sclerosis. Does anyone here have a story of morphea scleroderma. Unlike systemic scleroderma, localized scleroderma usually does not affect internal organs risk factors for localized scleroderma. Keloidal scleroderma is a very rare diagnosis, which has been also reported with alternate nomenclature including keloidal morphea, nodular morphea, and nodular scleroderma. Localized scleroderma morphea is a cutaneous limited fibrosis and represents a wide variety of clinical entities. Generalized morphea can prevent and mimic diffuse cutaneous ssc, but this clinical variant does not have raynauds phenomenon, digital. Treatment aims to relieve the symptoms, and will vary from person to person. Approximately 80% of all major kidney problems occur within the first 4 to 5 years of the disease. A type of light treatment called narrowband uvb treatment can be helpful. Localized scleroderma patients have a 10year survival rate of 75%.
Localised scleroderma causes one or more patches of hard skin. Generalized morphea is a subtype of localized scleroderma that lacks systemic manifestations. It can be clinically classified in different forms. I dont want to miss anything and want to try and circumvent more damage to my fairly young body. Localized fibrosing disorders include a spectrum of rare conditions that frequently begin in childhood. Localized scleroderma morphea like is a rare disease in dogs and extremely rare in cats and it is an uncommon chronic connective tissue disease. Patients with this rare form of localized scleroderma have the same skin problems as those with other types but can also develop large blisters called bullae or abrasions. Highseverity was defined as presentation with pansclerotic or generalized morphea, losf and subtypes with evidence of high morbidity e. Scleroderma foundation hello, i know there are a few here that have done a fat transfer due to asymmetry or cosmetic problems of their face.
Scleroderma renal crisis is much less common in limited scleroderma although it can occur, often early in the disease. Morphea occurs in childhood as well as in adult life. Localized scleroderma is different from systemic scleroderma, in that it only affects the skin, related tissues, and muscles below the tissues. The inflammation can trigger connective tissue cells to produce too much collagen, a fibrous protein that is a major part of many tissues. Morphea is an uncommon condition that is thought to affect 2 to 4 in 100,000 people. Morphea or localized scleroderma is a rare disorder with characteristic clinical features. Scleroderma can be classified into disease restricted to the skin localized scleroderma, including the morphea and linear forms and systemic disease with visceral organ involvement systemic sclerosis box 211. Fat transferinjection for morphea scleroderma on facejaw in. The prevalence rates of diseases that share scleroderma as a clinical feature are reported ranging from 4 to 253 cases per 1 million individuals.
It often affects the skin tissues and does not cause harm to the other major organs. Morphea is a term used to describe the skin symptoms of localized scleroderma, although the terms sometimes are used interchangeably. While it can occur in all age groups, it is more common among adults. These are followed by the appearance of firm, hard, ovalshaped plaques with ivory centers that are encircled by a violet ring.
Download the pdf read or download this clinical story in italian return to main page dr. 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. The treatment of this disease involves the use of long term immunomodulators. 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. Usually they appear on the trunk, but they can affect any part of the body. Localized scleroderma is an autoimmune disease in which the immune system causes inflammation in the skin. Voices of scleroderma volume 1 features juvenile scleroderma expert dr. Morphea scleroderma usually begins as patches of yellowish or ivorycolored rigid, dry skin. Recent studies show that the localized form may affect internal organs and have variable morbidity. Apr 16, 2018 morphea, also known as localized scleroderma, is an idiopathic inflammatory disorder that causes sclerotic changes in the skin. Scleroderma symptoms scleroderma education project. It causes aesthetic impairment due to the sclerotic skin and disability of varying degrees, particularly in the cases where the subcutaneous regions are involved as well. Up to three in every 100,000 people are diagnosed with localized scleroderma every year, and the disease is. For unknown reasons, serious kidney problems are more common in men and with patients who had an older age of disease onset.
Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. The scleroderma home treatment guide of naturopathic. Morphea localized scleroderma symptoms and prognosis see online here morphea or localized scleroderma is a rare disorder with characteristic clinical features. This rare autoimmune connective tissue disorder caused by the overproduction of collagen hardens skin and internal organs. Feb 15, 2018 morphea is a skin condition that causes patches of reddish skin that thicken into firm, ovalshaped areas. 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. Localized scleroderma skin lesions can get better or even go away. In children, linear morphea on the arms and legs can affect the underlying bone and interfere with bone growth. Types of scleroderma there are two main forms of scleroderma. Localized scleroderma is an uncommon condition, and affects approximately 20 people in every million.
Systemic sclerosis can be further subclassified based. Development of consensus treatment plans for juvenile. Mar 21, 2018 scleroderma can be classified into 2 broad categories, which include localized scleroderma and systemic scleroderma. Morphea, in fact, is only one particular type of localized scleroderma. The doctors told me it is rare to get internal scleroderma when you have morphea so hopefully the treatments will help. In a survey conducted in england and ireland, the annual incidence of juvenile ls was 3. For this, experts have said that even though people often use both these terms interchangeably, morphea is a specific form of localized scleroderma. Treatment of morphea localized scleroderma in adults uptodate. Morphea, also known as localized scleroderma, is an idiopathic, inflammatory disorder that causes sclerotic changes in the skin.
Morphea is relatively uncommon and women are affected about three times as often as men. Morphea is a form of scleroderma that is more common in women than men, in a ratio 3. Morphea is the name given to localised patches of hardened skin that are smooth and shiny. My youngest son, now 40 has had ra since age 5 and takes orencia. Skin is designed to be supple and smooth, and organs work best when there is no restriction or fibrosis. Morphea, also known as localized scleroderma, is an idiopathic inflammatory disorder that causes sclerotic changes in the skin. Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs. 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. A person with generalized morphea has patches in more than two parts of the body. It can cause swelling or pain in your muscles and joints. Until your condition clears up, you may want to pursue treatment that helps control your signs and symptoms. Symptoms may include areas of thickened skin, stiffness, feeling tired, and poor blood flow to the fingers or toes with cold exposure. Scleroderma systemic scleroderma, localized scleroderma.
Morphea, or localized scleroderma, usually begins between the ages of 20 to 50 years as patches of yellowish or ivorycolored rigid, dry skin inflammatory stage. The disease can be either localized to the skin or involve other organs in addition to the skin. Systemic sclerosis ssc is a multisystem and chronic disease characterized by abnormalities of small blood vessels and fibrosis of the skin and internal organs. The prognosis for those with scleroderma is highly variable and depends primarily on the form of the disease. The incidence of ls is reported to be approximately 27 cases1,000,000 1, 2. The manifestations of localized scleroderma are easily overlooked or attributed to other etiologies, particularly in the early stages table 282. Evidencebased treatment strategies in morphea are lacking. Generalized or pansclerotic morphea may pose more diagnostic dilemmas. Nandini moorthy, who wrote the medical overview of juvenile scleroderma. It also features many true stories of patients and caregivers of juvenile scleroderma, morphea, and linear scleroderma. Morphea is one of the categories of localized scleroderma 2 3. Most of the times, patients and their family members ask a common question whether morphea and scleroderma are same.
Both the incidence of morphea and linear scleroderma are more common in females, with the male to female ratio being approximately 3 to 4. Localized scleroderma is an inflammatory condition that causes hard and thickened patches of skin to form on different areas of the body. Localized scleroderma or morphea affects schoolaged children, is usually self limited and. Morphea is characterized by skin thickening with increased quantities of collagen in the indurative lesion. The treatment of this disease involves the use of longterm immunomodulators. Scleroderma is a disease of the bodys connective tissue.
Morphea is a localized form of scleroderma and affects primarily just the skin. Although uncommon, localized scleroderma occurs approximately 20 times more often than systemic sclerosis. Collagen is a protein normally present in our skin that provides structural support. Systemic sclerosis can be further subclassified based on the extent of involvement. Treatment of morphea localized scleroderma in adults. Disease activity typically persists for three to six years, although some patients develop more persistent or recurring episodes of activity. The linear type can cause problems to underlying tissues such as muscles and bones. A case of advanced systemic sclerosis with severe gerd. The patches vary in size and typically have a red border and a thickened paleyellow center. Morphoea american spelling, morphea is characterised by an area of inflammation and fibrosis thickening and hardening of the skin due to increased collagen deposition. There are two subtypes of this form of scleroderma. The condition is rare and thought to affect less than 3 out of. Morphea or localized scleroderma, is a benign, chronic, and autoimmune connective tissue disease, characterized by a hardening of the skin, due to an increased synthesis of collagen. Morphea subtypes are classified according to their clinical presentation and depth of tissue involvement.
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. In children, the linear subtype predominates while in adults plaque and generalized subtypes are most common. Morphea genetic and rare diseases information center gard. It is a rare condition in which idiopathic sclerosis of the skin occurs in a widespread manner. Uva1 produces long wavelengths that can deeply penetrate. German guidelines for the diagnosis and therapy of. The term scleroderma covers various types of morphoea and systemic sclerosis.
While this does not imply that patients with localized scleroderma evolve to develop these. These then become hard, slightly depressed, ovalshaped plaques which usually have a whitish or yellowish center surrounded by a pinkish or purplish halo. There are two types of localized scleroderma, namely morphea and localized linear scleroderma. Morphea occurs more frequently in women, especially in adults where female predominance is marked 5. Werth,md philadelphia, pennsylvania morphea is a rare. If morphea crosses a joint, the thickening may limit joint movement. Learn more about the clinical features, pathophysiology, laboratory diagnosis, treatment, and the prognosis. Update on the classification and treatment of localized scleroderma. Outcome measures and treatment nicolefett,md,andvictoriap. My 25 year old daughter was diagnosed last year with morphea localised scleroderma. Just remember some side effects are rare but you can be the one person who gets them. Morphea american osteopathic college of dermatology aocd. Systemic involvement in localized sclerodermamorphea. Jun 24, 20 morphea is characterized by skin thickening with increased quantities of collagen in the indurative lesion.
It is an excellent allaround book for patients and caregivers. Connective tissue is the material inside your body that gives your tissues their shape and helps keep them strong. Systemic involvement in localized sclerodermamorphea anna gorkiewiczpetkow, md, phd, agnieszka kalinskabienias, md, phd. This entity is subdivided into linear scleroderma, plaque. Localized scleroderma is characterized by thickening of the skin from excessive collagen deposits. The underlying pathogenesis of morphea is not completely understood at this time, but ultimately results in an imbalance of collagen production and. Systemic scleroderma tends to progress faster in men and. However morphea can occur in a generalized form as well as guttate, nodular, subcutaneous and linear forms. They are painless and there are normally no other problems or symptoms. 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 usually goes away without treatment, though it may leave scars or areas of discolored skin. Subtypes of morphoea vary according to the location. It is a form of scleroderma patches most often occur on the abdomen, stomach, and back, and sometimes on the face, arms and legs.
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