| Localized Scleroderma: Linear |
| This page was written by Shelley Ensz, and medically edited by Dolores Vázquez-Abad, M.D.. The sections on En Coup de Sabre and Parry-Rombergs have not been medically edited yet. See Disclaimer. |
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| What is Linear Scleroderma? |
| Linear Scleroderma is a line of thickened skin which can affect the bones and muscles underneath it, thus limiting the motion of the affected joints and muscles. It most often occurs in the arms, legs, or forehead, and may occur in more than one area. |
| Its most likely to be on just one side of the body. Linear Scleroderma generally appears in young children, and is characterized by the failure of one arm or leg to grow as rapidly as its counterpart. |
| Linear morphoea follows Blaschko's lines. Our data indicate that linear morphoea follows the lines of Blaschko. We hypothesize that in patients with linear morphoea susceptible cells are present in a mosaic state and that exposure to some trigger factor may result in the development of this condition. (PubMed) Br J Dermatol. 2008 Jul;159(1):175-81. (Also see: Morphea ) |
| Systemic Sclerosis and Localized Scleroderma in Childhood. Juvenile scleroderma syndromes, including the systemic and the localized varieties, represent the third most frequent chronic rheumatic conditions in pediatric rheumatology practice. (ScienceDirect) Rheumatic Disease Clinics of North America Vol 34, Issue 1, Feb 2008, Pp 239-255. (Also see: Juvenile Scleroderma ) |
| Generalized Linear Scleroderma in Childhood. A 6 year-old girl presented with linear hyperpigmented, indurated plaques on the upper and lower extremities of 9 months duration The onset followed a febrile illness that was diagnosed as typhoid. Dermatology Online Journal 13 (3): 34. 2007. (Also see: Juvenile Scleroderma ) |
| Congenital localized scleroderma (CLS). CLS is a rare and probably underestimated condition in neonates. CLS should be included in the differential diagnosis of infants with cutaneous erythematous fibrotic lesions to avoid functional and aesthetic sequelae and to allow prompt therapy. PubMed. J Pediatr. 2006 Aug;149(2):248-51. (Also see: Juvenile Scleroderma ) |
| Localized scleroderma is an autoimmune disorder. Many previous studies conclude that localized scleroderma involves autoimmune abnormalities and is one of the organ-specific autoimmune disorders targeting mainly skin, although the types of autoimmune abnormality are different from systemic sclerosis. PubMed. Rheumatology (Oxford). 2004 Nov 23. (Also see: Types of Scleroderma: Localized, Morphea and Dr. Shinichi Sato) |
| Unilateral generalized morphea (UGM) is a rare variant of localized scleroderma. As the onset of UGM usually occurs in pediatric patients, pediatricians should be cognizant of the presentation of this uncommon condition. PubMed. Eur J Med Res. 2006 Apr 28;11(4):152-6. (Also see: Localized Scleroderma: Morphea and Juvenile Scleroderma) |
| Is Juvenile Localized Scleroderma really "LOCALIZED"? One fourth of JLS patients in this data series presented various kind of extra-cutaneous manifestations, sometimes with multiorgan involvement. For this reason, the term "localized" is somehow inappropriate. These findings should change our clinical approach to this disease and underline the need for systemic immunosuppressive treatment for some patients. Francesco Zulian. ACR Conference Oct. 2003. (Also see: Types of Scleroderma and Morphea) |
| Juvenile localized scleroderma in the first year of life. JLS can start as early as during the first year of life and can be present even at birth. Linear subtype was the prevalent manifestation of the disease. Since the diagnostic delay was remarkable and the evolution quite aggressive, JLS should be considered in the differential diagnosis of infants with fibrotic or hyperpigmented skin lesions. Francesco Zulian. 1396/218. ACR 2004. |
| En Coup de Sabre |
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| Parry-Rombergs Syndrome |
| Parry-Rombergs Syndrome, is also known as Progressive Hemifacial Atrophy or HFA. This may be the same as Linear Scleroderma affecting the facial area. |
| En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. En coup de sabre morphea and Parry-Romberg syndrome frequently coexist and are likely both variants of morphea. Bilateral disease is more common than previously reported. The efficacy of antimalarials and methotrexate in the treatment of these diseases remains unclear. PubMed. J Am Acad Dermatol. 2007 Feb;56(2):257-63. (Also see: En Coup de Sabre ) |
| Neuroretinitis, Parry-Romberg syndrome, and scleroderma. The authors report a new case of progressive hemifacial atrophy (Parry-Romberg syndrome). They emphasize the rarity of this disease, its etiology, which remains controversial, the diversity of its ophthalmologic manifestations, and its relations with scleroderma and autonomic nervous system disorders are discussed. Acute neuroretinitis remains rare and its pathogeny is unknown. PubMed. J Fr Ophtalmol. 2005 Oct;28(8):866-70. |
| Beneficial effect of immunosuppressive drugs on Parry-Romberg syndrome. We observed beneficial effects of immunosuppressive agents on neurologic lesions in particular in a patient with PRS who presented with immunoinflammatory findings and neurologic involvement, apart from cutaneous manifestations. PubMed. South Med J. 2005 Sep;98(9):940-2. |
| Progressive hemifacial atrophy with linear scleroderma. We think that linear scleroderma of childhood and hemifacial atrophy have considerable clinical overlap and these two syndromes appear to be manifestations of the same or related pathogenic processes. Recently, the beneficial effects of 1.25-dihydroxyvitamin D3 (calcitriol) were reported in adults and in children with linear scleroderma. Our result indicates that calcitriol may be an effective agent for treating localized scleroderma in children. PubMed. Pediatr Dermatol. 2005 Sep-Oct;22(5):436-9. |
| Jessica G: Parry-Romberg Syndrome By the
time I was about five years old, the disease had already disfigured my
left side drastically. I have had over fifty operations and surgical procedures for Romberg's.... |
| Sclerodermic Linear Lupus Panniculitis |
| Sclerodermic linear lupus panniculitis: report of two cases. Lupus erythematosus panniculitis is a rare disease characterized by deep subcutaneous nodules, most commonly localized on the upper limbs and face. We present here the clinical characteristics, course and laboratory findings of 2 patients having linear lupus erythematosus panniculitis with localized scleroderma-like changes. PubMed. Dermatology. 2005;210(4):329-32. (Also see: Lupus, Linear Scleroderma, and Overlap Syndrome) |
| Diagnosis |
| Linear scleroderma is usually diagnosed by clinical examination. It is often confirmed by skin biopsy. Skin biopsies are usually a very tiny sample of tissue and the procedure is often very quick, easy and with minimal discomfort. Ultrasound has also been found to be useful for the diagnosis of Localized Scleroderma (such as linear). |
| Localized Scleroderma (LS) Severity Index and Global Assessments: A Pilot Study of Outcome Instruments. LS clinical trials are impeded by the lack of reliable and reproducible outcome measures. We have developed the LS Severity Index (LoSSI) to correct this deficiency. Journal of Rheumatology. Volume 35: No. 3 March 2008. |
| Plaque-type scleroderma associated with linear and oesophageal features and facial and extra-facial hemiatrophy. The combination of several sub-types of scleroderma and facial and extra-facial hemiatrophy in the same patient may indicate that these entities actually represent different spectra of the same disease. PubMed. Ann Dermatol Venereol. 2007 Jan;134(1):68-71. (Also see: Morphea ) |
| A new computerized method for the assessment of skin lesions in localized scleroderma (LS). CSS (computerized skin score) has shown to be a reliable method to assess the skin lesions in patients with LS. It is reproducible, easy to use and, with the support of the CSS software, applicable worldwide. PubMed. Rheumatology (Oxford). 2007 Jan 30. (Also see: Localized Scleroderma: Morphea ) |
| Ultrasonography (USG) is a Sensitive Tool for Monitoring Localized Scleroderma (LS). Changes seen on USG correlated with assessed clinical changes, both improvement and worsening. Using USG to monitor patients with LS may, therefore, be useful for guiding treatment strategies and as a tool in controlled clinical trials. Suzanne C. Li. 1699/370 ACR 2006. |
| Serum Autoantibodies and their Clinical Associations in Patients with Childhood and Adult Onset Linear Scleroderma. Childhood onset Linear Scleroderma is similar to adult onset disease in regard to the frequency of serum autoantibodies. Thaschawee Arkachaisri. 289/289 ACR 2006. (Also see: Antibodies ) |
| Serum Autoantibodies and their Clinical Associations in Patients with Childhood and Adult Onset Linear Scleroderma (LScl). Childhood onset LScl is similar to adult onset disease in regard to the frequency of serum aAbs. Over two thirds of LScl patients had ANA. Thaschawee Arkachaisri. 289/289 ACR 2006. (Also see: Antibodies ) |
| Antihistone antibodies (AHAs) in linear scleroderma variants. AHAs, which traditionally are markers for drug-induced lupus, may also be linked to linear scleroderma. The AHA titers may be related to the extent of involvement as well as disease activity. PubMed. Int J Dermatol. 2006 Nov;45(11):1296-9. (Also see: Antibodies ) |
| Rheumatoid factor isotypes in localized scleroderma. The presence of RF isotypes is one of the immunological abnormalities of localized scleroderma. IgM RF seemed to be most useful of these three factors to determine the severity of disease. PubMed. Clin Exp Dermatol. 2005 Jul;30(4):405-8. |
| Anti-DNA Topoisomerase II Alpha Autoantibodies in Localized Scleroderma. The present results indicate that anti-topo II alpha Ab is a major autoantibody in LSc, which is distinct from anti-topo I Ab in SSc. Furthermore, our results suggest that the high prevalence of anti-topo II alpha Ab in LSc and exclusive presence of anti-topo I Ab in SSc suggest that Abs to enzymes of the topoisomerase family are associated with fibrotic disorders including LSc and SSc. Ikuko Hayakawa. ACR Conference Oct. 2003 (Also see: Localized Scleroderma: Morphea and Antibodies) |
| Treatment |
| No treatment, medication or ointment has been proven by large-scale placebo-controlled studies to alter the course of Linear Scleroderma. A placebo-controlled study is still necessary to confirm the methotrexate/corticosteriod therapy; and, by any means, these are toxic therapies, particularly in children. |
| Pulsed high-dose corticosteroids combined with low-dose methotrexate in severe localized scleroderma. These data suggest that pulsed high-dose corticosteroids combined with orally administered low-dose methotrexate therapy is beneficial and safe in the treatment of patients with LS. This treatment regimen should especially be considered for severe forms of LS in which conventional treatments have failed. PubMed. Arch Dermatol. 2005 Jul;141(7):847-52. (Also see: Morphea) |
| Photodynamic therapy (PDT) in dermatology. A therapeutical benefit of PDT is also evident for inflammatory dermatoses like localized scleroderma, acne vulgaris and granuloma annulare. The benefits of PDT are the low level of invasiveness and the excellent cosmetic results after treatment. PubMed. Photodermatol Photoimmunol Photomed. 2005 Jun;21(3):142-9. (Also see: Morphea) |
| Phototherapy and photochemotherapy of sclerosing skin diseases. Two phototherapeutic modalitites are used for the treatment of sclerosing skin diseases, long-wave ultraviolet A and psoralen plus ultraviolet A (PUVA). PubMed. Photodermatol Photoimmunol Photomed. 2005 Jun;21(3):157-65. |
| Linear Patient and Caregiver Stories |
| AJ Miller: Linear Scleroderma I have had Linear Scleroderma since 1968, when I was 7 years old. I have never met anyone else with it... |
| Alicia: Linear Scleroderma My doctor said the people who are looking at me and seeing the scars are the ones who need healing, and that I should pray for them... |
| Alicia B: Localized Morphea with possible Parry Romberg Syndrome The doctor kept saying something had eaten away at all the fatty tissue and the muscles which left it just skin, blood, bone, and nerve so when you look at it, it looks like I have dirt on my face and a gaping hole to go with it... |
| Angel: Linear Scleroderma I have linear scleroderma. I have had it for a little over five years and I am only twelve... |
| Angela: Mother of Linear Scleroderma Patient We are in search of others who know how to handle the pain and muscle spasms in her legs... |
| Anna: Linear Scleroderma (Poland) I realised that it is not worthwhile to give up, even during the most difficult moments; it is necessary to fight and to believe that it will be better. If I had not believed I would not have been alive now... |
| Ariel: Linear Morphea Luckily, it only affects my skin, so I can still dance and play the piano... |
| Ariel M-S: Linear Morphea Within six months, the morphea had eaten away almost all of the fat on my arm and I have developed nerve and muscle pains that do not go away... |
| Ashlin: Localized Scleroderma It took me five months to realize that I needed to go to the doctor, and now I feel that I should not have waited so long... |
| Aurora: Linear Scleroderma I am always very tired, and I suffer from pain that will not go away no matter what I do... |
| (Español/Spanish) Aurora: Esclerodermia Lineal Estoy agotada continuamente, y padezco dolores que no se quita con nada... |
| Carolina: Linear Scleroderma As time went by it disseminated throughout my leg and reached my foot... |
| (Español/Spanish) Carolina: Esclerodermia Lineal Luego de multiples examenes, de reiteradas visitas a diferentes dermatólogos e inmunólogos diagnosticaron una escleroderma localizada de tipo lineal... |
| Catherine: Mother of Linear Scleroderma Patient Emma's linear scleroderma started on her left cheek at the age of 4 years and by the time she was eight that side of her face was quite deformed... |
| Charmaine C: Lichen Sclerosus and Scleroderma Soon multiple spots developed across my abdomen, with a few stray ones on my forearms and more on the left leg... |
| Chris S: Mother of Linear Morphea Patient Whatever it was, I knew it should not be there... |
| Christin: Linear Scleroderma My name is Christin. I am a twenty-year-old college student. I have had scleroderma for as long as I can remember... |
| Davie's Mom: Mother of Linear Scleroderma Patient Davie could be a candidate for skin grafts. If anyone has tried this I would love some input... |
| Debbie W: Mother of Linear Scleroderma Patient Because my daughter's face was being affected so severely with atrophy, hair loss, and partial loss of her eyebrow we began a treatment program... |
| Debra R: Mother of Emma, Linear Scleroderma Patient It is so good to have support and be able to ask questions and have them answered. Now I can explain to Emma what is going on... |
| Deedra: Linear Scleroderma In the course of two years the scleroderma spread from under my knee to the top of my thigh... |
| Desiraye: Linear Morphea When I was 14 years old, I spent the whole summer laying out in the sun. I developed a white patch that my mother thought was ringworm... |
| (Polski) Dorota: Twardzina linijna (coup de sabre) Moja historia z twardzina zaczela sie 15 lat temu i do dzisiaj (kiedy trafilam na strony serwisu o sklerodermii) nie zdawalam sobie sprawy z tego, jak powazna... |
| Ed: Father of a Linear Morphea Patient A series of medications, steroids and creams has been applied, but Erwin's case seemed to be worsening... |
| Edna S: Linear Scleroderma I am desperate because I know this is a deteriorating illness... |
| Emmi: Linear Scleroderma and Vitiligo My parents thought I was going to die, and doctors here in Finland did not really know what to do with me. There are not many scleroderma cases in Finland... |
| (Español/Spanish) Edna S: Esclerodermia Lineal Estoy desesperada por que sé que esto es una enfermedad deteriorante... |
| Erica: Mother of Daughter with Linear/Morphea Scleroderma She went there with one spot and now she has them all over both arms, one hand, her back, knee and thigh... |
| (Italiano) Eugenio: Sclerodermia Lineare lPremetto che mi rendo conto della difficoltà di rispondere ad una mail senza poter vedere il paziente ma, come padre, ho sentito il bisogno di scriverLe per sapere la Sua opinione... |
| Gina: Morphea or Linear Scleroderma I really try to put it out of my mind and be thankful that it is not systemic. But, every time I notice new areas turning color it really worries me... |
| Ginger: Generalized/Linear Scleroderma After analyzing all the test results and my medical records the neurologist attributed the muscle atrophy to the linear scleroderma... |
| Heather: Vitiligo, Hypothyroidism, and now Linear Morphea I am not too concerned about the other two conditions since there really is nothing you can do about the vitiligo, and the thyroid is under control. The morphea is what is bugging me... |
| Hope: Linear Morphea My mom took me to the doctor and for three months he treated it as ringworm... |
| Jan L: Linear Scleroderma and Vitiligo I was not ever overly concerned with my looks, so I really did not pay that much attention to it... |
| Jason: Linear Scleroderma I have recently been diagnosed with Raynaud's as well, have had gastro-intestinal difficulties more common to systemic forms, and have witnessed these lesions grow and deepen rather than decrease in size... |
| Jennifer M: Mom of Linear Morphea Patient Almost the minute that the doctor walked into the room she knew what it was. The first words out of her mouth were, "That's linear morphea"... |
| (Español/Spanish) Jessica: Madre de Paciente con Esclerodermia Localizada en Golpe de Sable Tengo una niña de 6 años de edad, le diagnosticaron Esclerodermia Lineal en golpe de sable desde los 3 años... |
| Jessica G: Parry-Romberg Syndrome By the
time I was about five years old, the disease had already disfigured my
left side drastically. I have had over fifty operations and surgical procedures for Romberg's.... |
| Jessica M.: Mother of Linear Morphea Patient About eight months ago my daughter developed a dark circle on her leg. It looked like a deep bruise, but we let it go. It never went away... |
| Judy C: Mother of a Daughter with Linear Morphea Scleroderma Three months ago, our oldest daughter, Alison, was diagnosed with Linear Morphea Scleroderma (Juvenile Scleroderma)... |
| Julie B: Mother of Linear/Morphea Scleroderma Patient Her right leg is badly affected with a lot of scarring and muscle wasting and also the leg length... |
| Kathy Gaither: Mother of Juvenile Scleroderma Patient I decided that I was going to turn our negative experience with scleroderma into something positive for us and for other parents... |
| (Dutch) Leon: Lineair Bij mij ontdekte men op 5 jarige leeftijd twee vlekjes op mijn linker onderarm... |
| Lancia: A Friend of a Morphea Patient (Italy) Some time ago a beloved friend of mine was diagnosed with scleroderma... |
| Lisa Kate McGowan: Linear Scleroderma When I was eighteen years old, I was diagnosed with linear scleroderma. It changed my life because it affected my left arm by leaving a big shiny scar... |
| Louise: Linear Scleroderma I got a bit of a fright when I read about scleroderma affecting the organs... |
| (Italiano) Lucia: Sclerodermias a Colpo di Sciabola La lesione c depressa, larga circa un centimetro,di coloro scuro, localizzata in posizone centrale sulla fronte... |
| Malen: Linear Scleroderma (Philippines) My symptoms included severe cramps in my right leg, where the pain is pushing me to my limits. My leg and arm on the right side of my body are smaller... |
| Mary B: Scleroderma En Coup de Sabre I feel like it is tearing my head open. Sometimes I get headaches, and I believe it is because of this illness... |
| (Español/Spanish) Mary B: Esclerodermia en Golpe de Sabre Por el contrario siento que se me está haciendo una enorme brecha en la cabeza. A veces me dan dolores de cabeza y creo que es por causa de esta enfermedad... |
| Maria R: Linear Scleroderma I am as healthy as I ever was, except the line on my forehead never disappeared, but it does not bother me in any way... |
| Martina: Linear Morphea (Ireland) I am twenty-four years old. I was diagnosed with linear morphea in September... |
| Mary-Charlotte: Juvenile Linear Scleroderma I am now fourteen years old, and I have been in remission for about six years. I no longer take methotrexate or occupational therapy... |
| Melanie W: Linear Morphea Scleroderma The only good thing about this disease is that it makes you a better person.... |
| Miguel: Linear Scleroderma I am twenty-seven years old and some months ago I went to see a doctor because I had a spot on my left leg... |
| (Español) Miguel: Esclerodermia Lineal Hace unos meses fui a ver a un médico porque tenía una mancha en mi pierna derecha, no me molestaba pero mi piel comenzaba a endurecerse en esa área... |
| (Italiano) Paola - Sclerodermia Lineare 3 anni fa ho visto sotto il malleolo del piede destro di mio figlio Giacomo, che allora aveva 8 anni, una macchia color... |
| Patty S.:Localized Scleroderma, Pulmonary Hypertension and Sjögren's I have quit my job and reduced my stress a hundred percent. I am doting on my children and my husband... |
| Rhea: Morphea The giant spot on my back started out as Morphea and then started gaining more qualities of Linear Scleroderma... |
| Rodriguez: Linear Scleroderma Little by little pigmentation is coming back into my leg and there are times I still feel joint pain but I have gotten through it... |
| Rubie: Linear Morphea Scleroderma I was referred to a dermatologist and was finally diagnosed with linear morphea at the age of four... |
| Sarah H: Linear Scleroderma When I was very young, two or three years old, my mom started to notice that the top of my scalp was changing... |
| Stacie: Linear Scleroderma I may have been incurable at four years old but it gave me an insight. I appreciate my life so much more than I would have thought. It could be worse... |
| Tami: Morphea Scleroderma I have gotten used to the stares everyone casts my way because in their eyes I am different. Unique, I guess... |
| Tammy: Mother of Son with Linear and Morphea Scleroderma Brandan has linear scleroderma on his right side and morphea on his chest pretty bad... |
| Tammy M: Linear Scleroderma and Vitiligo When I was first diagnosed there was no such thing as the Internet. I always felt that my family and I were the only ones in the world who knew what scleroderma was... |
| Terry E. (Mojoy the Clown): Morphea Profunda Linear Scleroderma My right lower leg started to swell and the ankle grew big. It hurt all the time... |
| (Italiano) Tommaso DM: Lineare Ho quasi 13 anni. Sedici mesi fa mi è stata diagnosticata la sclerodermia... |
| Tore: Linear Scleroderma However, as time went by, the patch extended, and the hardening, instead of going down, got worse, involving more and more parts of my leg, all the way to my knee... |
| (Italiano) Tore: Lineare La cute ma anche i tessuti sottocutanei risultavano induriti. In realtà sembrava che la pelle si fosse completamente attaccata all'osso, dal quale era impossibile smuoverla... |
| Tori: Localized Juvenile Scleroderma This is not the first rare disease that I have had... |
| Vanessa: Linear Scleroderma My older sister found a way to cover up the nasty disease on my forehead. Bangs! It was a great idea... |
| Yally: Dermatitis with Linear/Morphea My daughter has a spot on her left leg, that almost covers it completely... |
| (Español/Spanish) Yally: Dermatitis con Consistencia en Morfea/Lineal Mi temor siempre ha sido el consumir indefinidamente la droga... |
| See Also |
| Causes of Scleroderma. ISN. |
| En Coup de Sabre. Includes patient stories. ISN. |
| Juvenile scleroderma. Scleroderma is a relatively rare disorder in children. Among its subsets, localized scleroderma is more common in children than the systemic variety. Curr Opin Rheumatol 2002 Sep;14(5):553-61 PubMed. |
| Juvenile Scleroderma. ISN. |
| Parry-Rombergs Syndrome. Includes patient stories. Parry-Rombergs Syndrome. |
| Scleroderma Photos. Pictures of scleroderma symptoms, including photos of Linear Scleroderma. |
| Submit your Linear Scleroderma story in any language. ISN. |
