Systemic sclerosis can be further subclassified based. My 25 year old daughter was diagnosed last year with morphea localised scleroderma. 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. 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. The inflammation can trigger connective tissue cells to produce too much collagen, a fibrous protein that is a major part of many tissues.
Localized scleroderma is an autoimmune disease in which the immune system causes inflammation in the skin. Localized scleroderma is an uncommon condition, and affects approximately 20 people in every million. The localized scleroderma ls known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. I dont want to miss anything and want to try and circumvent more damage to my fairly young body.
Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. Apr 16, 2018 morphea, also known as localized scleroderma, is an idiopathic inflammatory disorder that causes sclerotic changes in the skin. Morphea is a term used to describe the skin symptoms of localized scleroderma, although the terms sometimes are used interchangeably. Morphea usually subsides on its own over time, though recurrences are common. These are followed by the appearance of firm, hard, ovalshaped plaques with ivory centers that are encircled by a violet ring. 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. Departmentofdermatology andimmunodermatology,medicaluniversity ofwarsaw,koszykowa 82astr,02 008warsaw,poland abstract localized scleroderma losc, also known as morphea, is a rare fibrosing disorder of. 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. It can cause swelling or pain in your muscles and joints. The common plaque type usually causes no problems apart from sometimes an unsightly appearance and tends to fade in time. Just remember some side effects are rare but you can be the one person who gets them.
Does anyone here have a story of morphea scleroderma. Morphoea american spelling, morphea is characterised by an area of inflammation and fibrosis thickening and hardening of the skin due to increased collagen deposition. Localized scleroderma is a rare disease of unknown etiology. Localized scleroderma is characterized by thickening of the skin from excessive collagen deposits. 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 treatment of this disease involves the use of long term immunomodulators. Feb 15, 2018 morphea is a skin condition that causes patches of reddish skin that thicken into firm, ovalshaped areas. Mar 21, 2018 scleroderma can be classified into 2 broad categories, which include localized scleroderma and systemic scleroderma.
Adequate studies on the incidence and prevalence have not been performed. Morphea usually goes away without treatment, though it may leave scars or areas of discolored skin. This involves hard, ovalshaped patches on the skin. Systemic scleroderma tends to progress faster in men and. The prevalence rates of diseases that share scleroderma as a clinical feature are reported ranging from 4 to 253 cases per 1 million individuals. Localized scleroderma is different from systemic scleroderma, in that it only affects the skin, related tissues, and muscles below the tissues. 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. Scleroderma symptoms scleroderma education project. Symptoms may include areas of thickened skin, stiffness, feeling tired, and poor blood flow to the fingers or toes with cold exposure. I would love to hear from others on here who either have morphea alone or had morphea and were then later diagnosed with systemic disease. Generalized or pansclerotic morphea may pose more diagnostic dilemmas. 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. Morphea is a localized form of scleroderma and affects primarily just the skin.
Morphea is distinct from systemic sclerosis scleroderma, an autoimmune connective tissue disorder characterized by acral or diffuse cutaneous sclerosis and frequent systemic manifestations. It also features many true stories of patients and caregivers of juvenile scleroderma, morphea, and linear scleroderma. The disorder can be mild, moderate or severe, depending on how much skin and connective tissue is affected. A case of advanced systemic sclerosis with severe gerd. Recent studies show that the localized form may affect internal organs and have variable morbidity. It can be clinically classified in different forms. If morphea crosses a joint, the thickening may limit joint movement. Disease activity typically persists for three to six years, although some patients develop more persistent or recurring episodes of activity. Until your condition clears up, you may want to pursue treatment that helps control your signs and symptoms. Classification of morphea localized scleroderma sciencedirect. Localized scleroderma is a skin condition that can take many. The term scleroderma covers various types of morphoea and systemic sclerosis. 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. Morphea is diagnosed based on findings of skin examination and skin biopsy.
Morphea localized scleroderma symptoms and prognosis. 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. The scleroderma home treatment guide of naturopathic. Werth,md philadelphia, pennsylvania morphea is a rare. Types of scleroderma there are two main forms of scleroderma. The manifestations of localized scleroderma are easily overlooked or attributed to other etiologies, particularly in the early stages table 282. Localized scleroderma skin lesions can get better or even go away. While it can occur in all age groups, it is more common among adults.
Abstract morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. Morphea is one of the categories of localized scleroderma 2 3. Morphea is a fibrosing disorder of the skin without systemic involvement. Systemic involvement in localized sclerodermamorphea anna gorkiewiczpetkow, md, phd, agnieszka kalinskabienias, md, phd. My youngest son, now 40 has had ra since age 5 and takes orencia. Morphea, also known as localized scleroderma, is an idiopathic inflammatory disorder that causes sclerotic changes in the skin. Usually they appear on the trunk, but they can affect any part of the body. Scleroderma is a disease of the bodys connective tissue. Localized scleroderma patients have a 10year survival rate of 75%. Up to three in every 100,000 people are diagnosed with localized scleroderma every year, and the disease is. Based on clinical morphologic findings, morphea can be classified into five groups.
A type of light treatment called narrowband uvb treatment can be helpful. There are two subtypes of this form of scleroderma. Morphea is a form of scleroderma that is more common in women than men, in a ratio 3. Treatment of morphea localized scleroderma in adults uptodate. The prognosis for those with scleroderma is highly variable and depends primarily on the form of the disease. 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 or morphea affects schoolaged children, is usually self limited and. Most of the times, patients and their family members ask a common question whether morphea and scleroderma are same. Jul 07, 2017 morphea is a skin condition that involves a patch or patches of discolored or hardened skin on the face, neck, hands, torso, or feet. Scleroderma systemic scleroderma, localized scleroderma. However, when too much collagen is made, the skin becomes stiff and hard. Treatment of morphea localized scleroderma in adults.
Both the incidence of morphea and linear scleroderma are more common in females, with the male to female ratio being approximately 3 to 4. 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 disease can be either localized to the skin or involve other organs in addition to the skin. In children, linear morphea on the arms and legs can affect the underlying bone and interfere with bone growth. A person with generalized morphea has patches in more than two parts of the body. Download the pdf read or download this clinical story in italian return to main page dr. Systemic sclerosis ssc is a multisystem and chronic disease characterized by abnormalities of small blood vessels and fibrosis of the skin and internal organs. Morphea or localized scleroderma is a rare disorder with characteristic clinical features. Approximately 80% of all major kidney problems occur within the first 4 to 5 years of the disease. Localized scleroderma can be divided into five general subtypes. The cochrane library, medline and embase from inception until may of 2011 were searched using the key words morphea and morphea treatment. The treatment of this disease involves the use of longterm immunomodulators.
Ssc, when advanced, is often compromised with severe gastroesophageal reflux disease gerd, which may be lethal in a worstcase scenario. These lesions are characterized by circumscribed fibrotic areas involving different levels of the dermis, subcutis, and, sometimes, underlying soft tissue and bone. Localized scleroderma is an inflammatory condition that causes hard and thickened patches of skin to form on different areas of the body. Morphea scleroderma usually begins as patches of yellowish or ivorycolored rigid, dry skin. Nandini moorthy, who wrote the medical overview of juvenile scleroderma. Unlike systemic scleroderma, localized scleroderma usually does not affect internal organs risk factors for localized scleroderma. Localized fibrosing disorders include a spectrum of rare conditions that frequently begin in childhood. Localised scleroderma causes one or more patches of hard skin.
Morphea is characterized by patches of yellowish or ivorycolored rigid, dry skin that become hard, slightly depressed oval plaques. The topic localized scleroderma morphea you are seeking is a synonym, or alternative name, or is closely related to the medical condition morphea. Although uncommon, localized scleroderma occurs approximately 20 times more often than systemic sclerosis. Fat transferinjection for morphea scleroderma on facejaw in. Morphea is characterized by skin thickening with increased quantities of collagen in the indurative lesion. Morphea is relatively uncommon and women are affected about three times as often as men. German guidelines for the diagnosis and therapy of.
The condition is rare and thought to affect less than 3 out of. However morphea can occur in a generalized form as well as guttate, nodular, subcutaneous and linear forms. Development of consensus treatment plans for juvenile. There are two types of localized scleroderma, namely morphea and localized linear scleroderma. Systemic sclerosis can be further subclassified based on the extent of involvement. Scleroderma is a chronic condition that affects the connective tissue. Morphea is the name given to localised patches of hardened skin that are smooth and shiny. It often affects the skin tissues and does not cause harm to the other major organs. Update on the classification and treatment of localized scleroderma. Localized scleroderma an overview sciencedirect topics. Uva1 produces long wavelengths that can deeply penetrate. Morphea genetic and rare diseases information center gard.
Localized types of scleroderma are those limited to the skin and related tissues and, in some cases, the. Generalized morphea is a subtype of localized scleroderma that lacks systemic manifestations. Scleroderma nord national organization for rare disorders. Evidencebased treatment strategies in morphea are lacking. It is a rare condition in which idiopathic sclerosis of the skin occurs in a widespread manner. Morphea is a localized type of scleroderma that is characterized by thickened skin patches, which can increase and decrease in size. While this does not imply that patients with localized scleroderma evolve to develop these.
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. Learn more about the clinical features, pathophysiology, laboratory diagnosis, treatment, and the prognosis. Evidencebased recommendations for treatment fett nm. Skin is designed to be supple and smooth, and organs work best when there is no restriction or fibrosis. Morphea occurs more frequently in women, especially in adults where female predominance is marked 5. 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. In a survey conducted in england and ireland, the annual incidence of juvenile ls was 3. In this article, we will learn about the clinical features including the di. 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. Lesions are usually limited and most commonly just one lesion is found. The linear type can cause problems to underlying tissues such as muscles and bones. Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs.
An association has been found with a parasitic disease toxoplasma. It is a form of scleroderma patches most often occur on the abdomen, stomach, and back, and sometimes on the face, arms and legs. Generalized morphea can prevent and mimic diffuse cutaneous ssc, but this clinical variant does not have raynauds phenomenon, digital. Keloidal scleroderma is a very rare diagnosis, which has been also reported with alternate nomenclature including keloidal morphea, nodular morphea, and nodular scleroderma. Morphea subtypes are classified according to their clinical presentation and depth of tissue involvement. Morphea usually occurs on the trunk, although it may be widespread generalized morphea. Scleroderma renal crisis is much less common in limited scleroderma although it can occur, often early in the disease. Systemic scleroderma occurs throughout the body, affecting internal organs. The underlying pathogenesis of morphea is not completely understood at this time, but ultimately results in an imbalance of collagen production and. Localized scleroderma morphea is a cutaneous limited fibrosis and represents a wide variety of clinical entities. The incidence of ls is reported to be approximately 27 cases1,000,000 1, 2. For this, experts have said that even though people often use both these terms interchangeably, morphea is a specific form of localized scleroderma. It is a group of diseases that cause abnormal growth of connective tissue.
A rare association of localized scleroderma type morphea. Morphea occurs in childhood as well as in adult life. In children, the linear subtype predominates while in adults plaque and generalized subtypes are most common. Voices of scleroderma volume 1 features juvenile scleroderma expert dr.
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. Highseverity was defined as presentation with pansclerotic or generalized morphea, losf and subtypes with evidence of high morbidity e. These then become hard, slightly depressed, ovalshaped plaques which usually have a whitish or yellowish center surrounded by a pinkish or purplish halo. 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. This entity is subdivided into linear scleroderma, plaque. Morphea morphea consists of patches of thickened skin that can vary from half an inch to six inches or more in diameter. 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, 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. It is an excellent allaround book for patients and caregivers. Subtypes of morphoea vary according to the location. Jun 24, 20 morphea is characterized by skin thickening with increased quantities of collagen in the indurative lesion. This rare autoimmune connective tissue disorder caused by the overproduction of collagen hardens skin and internal organs.
For unknown reasons, serious kidney problems are more common in men and with patients who had an older age of disease onset. When the morphea goes deeper, uva1 phototherapy may be prescribed. Connective tissue is the material inside your body that gives your tissues their shape and helps keep them strong. 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. They are painless and there are normally no other problems or symptoms. Morphea, also known as localized scleroderma, is an idiopathic, inflammatory disorder that causes sclerotic changes in the skin. Apr 03, 2012 localized scleroderma is characterized by thickening of the skin from excessive collagen deposits. Morphea american osteopathic college of dermatology aocd.
The patches vary in size and typically have a red border and a thickened paleyellow center. Morphea is an uncommon condition that is thought to affect 2 to 4 in 100,000 people. Patients present with single or multiple inflammatory or sclerotic plaques. Morphea is not the same as scleroderma systemic sclerosis. Localized scleroderma genetic and rare diseases information. Treatment aims to relieve the symptoms, and will vary from person to person. Systemic involvement in localized sclerodermamorphea.
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