Localized Scleroderma: Linear |
| This page was written by Shelley Ensz, and has not yet been medically edited. See Disclaimer. |
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| Overview of 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. (Also see: Types of Scleroderma) |
| Localized Fibrosing Disorders - Linear Scleroderma, Morphea, and Regional Fibrosis. Localized fibrosing disorders include several clinical and histopathological conditions that are similar to the skin involvement of systemic sclerosis, but the systemic features are absent. Localized fibrosing disorders can be classified into several subtypes that include morphea, generalized morphea, and linear scleroderma. Mariana J Kaplan, MD. eMedicine. 12/03/08. |
| Autoimmune Disorder |
| 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. Rheumatology (Oxford). Vol 44. No. 3. 2004 Nov 23. (Also see: Types of Scleroderma: Localized, Morphea and Dr. Shinichi Sato) |
| Blaschko's Lines |
| 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) |
| Congenital and Neonatal Linear Scleroderma (Newborns and Infants) |
| 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) |
| Diagnosis of Linear Scleroderma |
| Diagnosis of Linear Scleroderma is usually done 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). ISN. |
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| Juvenile (Childhood) Scleroderma |
| Juvenile Scleroderma. When any form of scleroderma (either localized or systemic) occurs in children, it is also called Childhood Scleroderma or Juvenile Scleroderma. ISN. |
| En Coup de Sabre |
| En Coup de Sabre is a term used when linear scleroderma affects the forehead. It appears as an indented, vertical, colorless, line of skin on the forehead. The indentation looks as though the person may have been struck by a sword. ISN. |
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| Parry Rombergs Syndrome |
| Parry Rombergs Syndrome, is also known as Progressive Hemifacial Atrophy or HFA. It may be the same as Linear Scleroderma affecting the face. ISN. |
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| 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) |
| Treatments |
| Laparoscopic Sleeve Gastrectomy for Linear Scleroderma. This article documents the case of a 45-year-old female with a 35-year history of unilateral linear scleroderma, who subsequently developed morbid obesity of her contralateral side. Jason Kasza, MD. (Liebertonline). Journal of Laparoendoscopic & Advanced Surgical Techniques. May 2010, 20(4): 355-357. |
| Treatment of Pediatric Localized Scleroderma: Results of a Survey of North American Pediatric Rheumatologists (PR). Most PR in North America treat localized scleroderma with a combination of MTX and corticosteroids. However, there is no consensus on specific treatment regimens. There is a need for controlled treatment trials to better determine optimal therapy for this potentially disabling disease. SC Li. J Rheumatol 2009 Nov 16. (Also see: Juvenile Scleroderma and Medications) |
| Long Term Outcome of 14 Patients with Severe Form of Juvenile Localized Scleroderma (JLS). There are no accurate data on the natural history and optimal management of JLS. Methotrexate appears to be effective therapy in our cohort of patients with severe form of JLS and we propose a larger, multicenter study investigating impact of treatment on the long term outcome of JLS. T. Avcin. (SAT0541) EULAR 2009. (Also see: Juvenile Scleroderma and Medications) |
| Multiphoton laser scanning microscopy (MPLSM) of localized scleroderma. MPLSM could discriminate the sclerodermatous skin from the normal skin. With the advent of the clinical portability of typical MPLSM, this technique has great potential for application in the in vivo diagnosis of LS as well as for monitoring the treatment response. Lu, Kecheng. Skin Research and Technology, Volume 15, Number 4, November 2009 , pp. 489-495(7). |
| Characterization of the Cellular Infiltrate of Localized Scleroderma (LS) skin Lesions Before and After Methotrexate (MTX) or Prednisone Treatment. Juvenile LS active lesions are characterized by infiltration of CD8+ T cell and B cells, which seem to play an important role in the early phases of the disease. MTX treatment significantly reduces the inflammatory infiltrate, re-establish the CD4/CD8 T-cell ratio and induce the B-cell disappearance. F. Lunardi. (SAT0002) EULAR 2009. (Also see: Methotrexate) |
| Systemic and localized scleroderma in children : current and future treatment options. The juvenile forms of systemic sclerosis and localized scleroderma are important conditions in children because of the clinical severity and substantial mortality of systemic scleroderma and the major growth defects associated with childhood-onset localized disease even if the active disease itself is self-limited. For localized disease, anti-inflammatories, vitamin D analogs, and UV irradiation have been investigated. PubMed. Paediatr Drugs. 2006;8(2):85-97. |
| Improving Hand Function in Childhood Scleroderma using Continuous Passive Motion (CPM). Hand/wrist function in a highly motivated adolescent with localized scleroderma improved with the addition of CPM to her therapeutic regimen. Kimberly A. Wesdock. 1963/662 ACR 2006. (Also see: Sclerodactyly) |
| Treatment of Pediatric Localized Scleroderma with Methotrexate. Methotrexate appears to be a safe and effective therapy for pediatric Localized Scleroderma. S. J Rheumatol 2006; 33:609–14. (Also see: Localized Scleroderma) |
| 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... |
| More Linear Patient and Caregiver Stories... |
| See Also |
| Causes of Scleroderma. ISN. |
| Juvenile Scleroderma. ISN. |
| Scleroderma Photos. Pictures of scleroderma symptoms, including photos of Linear Scleroderma. |
| Submit your Linear Scleroderma story in any language. ISN. |
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