The treatment of this disease involves the use of long term immunomodulators. I think you should check this out with one of the volunteers there. It also features many true stories of patients and caregivers of juvenile scleroderma, morphea, and linear scleroderma. Solitary morphea profunda following trauma sustained in an. In contrast to morphea, linear scleroderma can affect the underlying bones and muscles. Morphea is classified according to the localization of the lesions and the depth of tissue involvement into localized or circumscribed limited to one or.
Morphea is a form of scleroderma that is more common in women than men, in a ratio 3. Morphea or localized scleroderma is a rare disorder with characteristic clinical features. Voices of scleroderma volume 3 features juvenile scleroderma expert dr. Linear morpheainduced atrophy treated with hyaluronic acid filler injections adrienne n. Morphea is divided into subgroups that are based on clinical appearance and distribution.
Patients with other indurated skin disorders such as scleromyxedema or scleredema adultorum. Systemic involvement in localized sclerodermamorphea. Histopathological changes in morphea and their clinical. Linear scleroderma definition of linear scleroderma by. Treatment of morphea localized scleroderma in adults uptodate. Suggest treatment for linear morphea healthcaremagic. Werth,md philadelphia, pennsylvania morphea is a rare. This report highlights excessive mucin deposition in lesions of morphea. The most frequent complications of morphea include arthralgias, uveitis, and joint contractures, especially in the linear and. 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.
Jul 31, 20 dermatopathology reference describes inflammatory morphea histopathology including histologic features and provides links to additional medical references. It most commonly affects the trunk but also occurs on the face and extremities. This classification is based on the morphologic findings and simplify the diagnostic and therapeutic approach 4, 5. Patients with other forms of morphea such as plaque morphea or linear morphea. Morphea pictures, symptoms, diagnosis and treatment. Frequent atrophy of adnexal structures, increased fibroblasts, thickening and luminal narrowing of small vessels, blunting of dermalsubcutis. Morphea genetic and rare diseases information center gard. Morphea, also known as localized scleroderma, is an idiopathic, inflammatory disorder that causes sclerotic changes in the skin. As the 20th century progressed, morphea studies mostly focused on clinical manifestations, histopathology, and classification.
Linear morphea with secondary mucinosis khandpur s, singh s. Treatment of morphea localized scleroderma in adults. Distinctive histopathologic findings in linear morphea en. Thickening and hyalinization of connective tissue of deep dermis, subcutaneous fat and muscular fascia, with perivascular and focal interstitial lymphocytic and plasma cell infiltrate in subcutaneous fat. This is characterised by atrophy and a furrow of the skin. Differential diagnosis of similar conditions along the lines of blaschko must be differentiated from linear le. There they diagnosed me with linear morphea and i was prescribed topical creams. When linear morphea is used more literally, it means that there is a mixture of both types linear and morphea, occurring together in combination. There is a sparse perivascular lymphocytic infiltrate with rare plasma cells and a hypocellular, sclerotic dermis. Treatment for linear morphea in child scleroderma inspire.
Solitary morphea profunda smp is a variant of localized scleroderma ls. I would keep a possibility of linear morpheascleroderma. When involving the scalp and face, linear morphea has been referred to as morphea en coup dsabre linear scleroderma can produce a scarred appearance on the face and scalp similar to what might result from the strike of a sword. Adultonset linear discoid lupus erythematosus on the. Morphea occurs in childhood as well as in adult life. Mar 23, 2020 morphea or localized scleroderma is a rare disorder with characteristic clinical features. The sclerodermic condition is marked by formation of hard plaques on the skin as well as hypopigmentation. Morphea is an uncommon condition that is thought to affect 2 to 4 in 100,000 people. Progressive enlargement of the plaques, however, can be debilitating as it leads to muscle atrophy. He has it from his toes on his left foot all the way up his leg and into his lower back. It presents as one or a few 34 patches of skin thickening with different degrees of pigment changes.
The lesions are firm but not hard and are usually depressed. Disease activity typically persists for three to six years, although some patients develop more persistent or recurring episodes of activity. Scleroderma was divided into morphea guttate type, linear scleroderma, generalized morphea, hemiatrophy, and. Adequate studies on the incidence and prevalence have not been performed. May 21, 2018 morphea, also known as localized scleroderma, is a disorder characterized by excessive collagen deposition leading to thickening of the dermis, subcutaneous tissues, or both. Morphea has an estimated annual population incidence of 2.
Linear and deep panslerotic morphea may cause considerable morbidity, especially in children, interfering with growth. Morphea is a skin condition that causes patches of reddish skin that thicken into firm, ovalshaped areas. Morphea localized scleroderma symptoms and prognosis. Linear scleroderma is an inflammatory disease of the dermis and subcutaneous tissue. Linear scleroderma is a variant of localized scleroderma in which sclerotic areas of skin develop in a linear pattern. Feb 15, 2018 linear morphea usually lasts longer, but also eventually improves. Linear morphea with secondary mucinosis khandpur s, singh. Morphea genetic and rare diseases information center. The condition is rare and thought to affect less than 3 out of. Clinical and histopathologic comparison of generalized. Morphea is classified into circumscribed, generalized, linear, and pansclerotic subtypes according to the clinical presentation and depth of tissue involvement. I am not sure that mino is good for one so little as it can discolour the teeth. This girl is a walking encyclopidia whose dad was a veterinarian so she learned a lot of medicine from him so go to. Learn more about the clinical features, pathophysiology, laboratory diagnosis, treatment, and the prognosis.
Good response of linear scleroderma in a child to ciclosporin. Morphea is localized scleroderma confined to the skin. Morphea, also known as localized scleroderma, is an idiopathic inflammatory disorder that causes sclerotic changes in the skin. Linear morphea definition of linear morphea by medical. Orringer,md the authors have indicated no significant interest with commercial supporters. Soeprono teaches and practices dermatopathology at loma linda university, school of medicine, department of dermatology. It is a form of scleroderma patches most often occur on the abdomen, stomach, and back, and sometimes on the face, arms and legs. Thus, sometimes the term linear morphea refers to localized scleroderma of any type either linear or morphea.
Soepronos textbook is available on and includes detailed information on over 600 entities and includes four dvd diskettes with highresolution images that provide a virtual dermatopathology reference and guide. Morphoea american spelling, morphea is characterised by an area of inflammation and fibrosis thickening and hardening of the skin due to increased collagen deposition. Although, the final diagnosis of cle was achieved by histopathological examination, dermatoscopic findings also play an important role to identify the accurate diagnosis. It has spread across the back and front of my shoulders, down the whole right side of my back, and now my right arm.
Morphea is distinct from systemic sclerosis scleroderma, an autoimmune connective tissue disorder characterized by acral or diffuse cutaneous sclerosis and frequent systemic manifestations. We report the case of a 50yearold white woman with a history of trauma sustained in an automobile accident who presented with smp on the right upper arm. Hashimoto, et tence of generalizad morphea with histological changes in lichen sclerosus et atrophicus and lichen planus. Further progression of neurological symptoms led to a caesarean section with the delivery of a healthy. The two main types are systemic scleroderma and localized scleroderma. In this case, the scleroderma is localized and unilateral, particularly in young children. Generalized morphea represents a rare subtype of the disease, which ranges from 3.
Systematically describe histologic changes of morphea in a large, well annotated cohort and determine the association between histopathology. The main subtypes are plaque morphea, linear scleroderma, generalized morphea, and pansclerotic morphea. The term scleroderma covers various types of morphoea and systemic sclerosis. Histopathological features in morphea localized scleroderma and their clinical correlates are poorly described. The histopathology of alopecia in linear morphea is typically. The underlying pathogenesis of morphea is not completely understood at this time, but ultimately results in an imbalance of collagen production and.
Presenting in the first decade of life, it involves the extremities either as a linear erythematous streak or as coalescing morpheaform plaques extending longitudinally to form a scarlike band fig. The histopathology of alopecia in linear morphea is typically characterized by sclerosis and a reduction in the number of follicular units. Morphea is a skin condition that involves a patch or patches of discolored or hardened skin on the face, neck, hands, torso, or feet. My son was diagnosed with linear morphea almost a year ago, however he likely had it for quite some time before it was diagnosed. Linear morpheainduced atrophy treated with hyaluronic acid. I was told that the diesase would stop spreading around the age of fifteen. Have you read the information available on the roadback site. Fernanda falcini of italy, who wrote the medical overview of juvenile scleroderma. Histopathology showed features of scleroderma with abundant mucin deposition in the reticular dermis. Feb 10, 2015 i would keep a possibility of linear morphea scleroderma. We sought to systematically describe histologic changes of morphea in a large, wellannotated cohort and determine the association between histopathology and clinical features. Linear morphea occurs in a line usually on the head, arm or leg.
A morphea this is the most common type of localized scleroderma. Preand immediate posttreatment images at the b treatment site demonstrating disease progression has occurred over 3 months. This report highlights excessive mucin deposition in. I am not 18 years old and the disease is still spreading. Linear cutaneous lupus erythematosus le is an unusual form of lespecific cutaneous condition, occurring in children and young adults. Subtypes of morphoea vary according to the location. Joint contractures, limblength discrepancy, and prominent facial atrophy shrinkage of underlying tissues can cause substantial disability and deformity. Patients present with single or multiple inflammatory or sclerotic plaques. The skin may be thickened, hard, and rigid, and pigmented patches may occur. Sometimes morphea is used as a generic term for localized scleroderma of any type. Clinical subtypes included circumscribed, linear, generalized, profunda, and morphea with lichen sclerosus features as previously defined.
Llinear morphea with secondary mucinosisinear morphea. It is an excellent allaround book for patients and caregivers. Jul 31, 20 dermatopathology reference describes morphea histopathology including histologic features and provides links to additional medical references. 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.
It presents as a linear band of hard bound, sclerotic skin, extending vertically on the forehead and extending variably into the scalp with. Outcome measures and treatment nicolefett,md,andvictoriap. We describe a 7yearold boy with progressive, linear, bounddown plaques involving the thighs, lower abdomen and back with no systemic involvement. If morphea crosses a joint, the thickening may limit joint movement.
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