| Juvenile Scleroderma |
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| What is Juvenile Scleroderma |
| There are two main types of Scleroderma: Localized and Systemic. |
| When scleroderma of any type afflicts children, it is called Juvenile Scleroderma. The localized forms of scleroderma (such as Linear and Morphea) are most common in children. |
| Please see Types of Scleroderma and/or consult with your doctor if you have any doubt about which form of scleroderma your child has. |
| 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 and Localized) |
| 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: Linear Scleroderma) |
| Systemic sclerosis in childhood. Juvenile systemic sclerosis is a rare disease of childhood, and the amount of published data is limited. It appears that its clinical presentation differs from adult disease and the limited form affects only very few children. PubMed. Rheumatology (Oxford). 2006 Oct;45 Suppl 3:iii28-iii29. |
| Classification in Systemic Sclerosis. Accurate classification of systemic sclerosis (SSc) has been an evolving issue in both pediatric and adult rheumatology literature. The need for classification criteria has been long recognized as a necessity for scientific inquiry, as SSc is a heterogeneous disease with variable expression, and prognosis is dependent on disease severity and target organ involvement. J Rheumatol 2006 May;33:840 Editorial. (Also see: Types of Scleroderma) |
| Juvenile Scleroderma Experts |
| Scleroderma Experts: US and worldwide listings. Only some of the expert centers treat children, and even fewer specialize in juvenile scleroderma. Please call the center(s) you are interested in for more information. ISN. |
| Italy: International Pediatric Center for Juvenile Scleroderma (both localized and systemic.) ISN. |
| New Jersey: Dr. L. Nandini Moorthy, Assistant Professor of Pediatrics at UMDNJ/Robert Wood Johnson Medical School. Featured expert on Juvenile Scleroderma in the ISN's book, Voices of Scleroderma Volume 2. ISN. |
| New York: Dr. Thomas Lehman, Chief of the Division of Pediatric Rheumatology at the Hospital for Special Surgery, Professor of Clinical Pediatrics at Cornell University Medical Center. Featured expert on Juvenile Scleroderma in the ISN's book, Voices of Scleroderma Volume 1. ISN. |
| Juvenile Scleroderma Books |
| Juvenile scleroderma experts whom we have featured in our Voices of Scleroderma book series include: |
| Voices of Scleroderma Volume 1: Dr. Thomas Lehman, New York. |
| Voices of Scleroderma Volume 2: Dr. L. Nandini Moorthy, New Jersey. |
| Voices of Scleroderma Volume 3: Dr. Fernanda Falcini, Italy. |
| All of these books are available on Amazon, and each one features a chapter of personal stories by juvenile scleroderma patients and caregivers, including an overview of juvenile scleroderma by world experts. We highly recommend any or all of these books, for well-rounded information and support. |
| Research about Juvenile Scleroderma |
| Mortality Outcomes in Pediatric Rheumatology Conditions. In the first systematic study of mortality rates in pediatric rheumatology the mortality rate for new patients seen in the U.S. during the 1990s was not increased in relation to the general population. The mortality rate was significantly increased in SLE, other connective tissue diseases, malignancies and systemic JRA. Philip J. Hashkes 254/254 ACR 2007. |
| Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. This study provides information on the largest collection of patients with juvenile SSc ever reported. Juvenile SSc appears to be less severe than in adults because children have less internal organ involvement, a less specific autoantibody profile, and a better long-term outcome. PubMed. Arthritis Rheum. 2006 Dec;54(12):3971-8. |
| Juvenile onset systemic sclerosis: a single center experience of 23 cases from Asia. DSSc and LSSc in childhood have a clinical presentation similar to adult patients, with cardiopulmonary involvement being the major predictor of outcome. The short-term prognosis of JSSc is good. PubMed. Clin Rheumatol. 2006 Dec 19. |
| 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) |
| Evaluation
of methotrexate and corticosteroids for the treatment of
localized scleroderma (morphea) in children. These data
suggest that systemic corticosteroids and methotrexate in
combination are beneficial and well tolerated in the treatment
of children with LS. Long-term monitoring is mandatory. IngentaConnect.
Br J Dermatol. 2006 Nov;155(5):1013-20. (Also see: Morphea) |
| 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: Linear Scleroderma) |
| Childhood Onset Systemic Sclerosis (SSc): Classification, Clinical and Serologic Features, and Survival in Comparison with Adult Onset Disease. Patients with juvenile onset SSc more frequently have an overlap syndrome with PM-DM, higher frequency of skeletal muscle involvement, serum anti-PM-Scl and anti-U1RNP antibody, fatal cardiac disease, and improved survival compared with adult onset SSc cases. PubMed. J Rheumatol. 2006 Apr 1. |
| 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. |
| 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) |
| Inflammatory myopathies in childhood: correlation between nailfold capillaroscopy findings and clinical and laboratory data. Nailfold capillaroscopy is an important tool for the diagnosis and follow-up of patients with rheumatic diseases, in particular dermatomyositis and scleroderma. A relationship has been observed in adults between improved capillaroscopic findings and reduced disease activity. PubMed. J. Pediatr (Rio J). 2006 January/February;82(1):40-45. (Also see: Dermatomyositis, and Raynaud's Diagnosis) |
| A Prospective Clinical and Pathological Study on Efficacy of Methotrexate (MTX)/Corticosteroid in Juvenile Localized Scleroderma (JLS). These preliminary results seem to confirm the efficacy of MTX in the treatment of JLS, especially if established early in the disease course. A good correlation between clinical improvement, thermography analysis and histological changes was found. Davide Meneghesso. 1411/187. ACR 2005. |
| Juvenile Systemic Sclerosis: A Follow-up Study of Eight Patients. Our results suggest that in jSSc, Raynaud's phenomenon is more severe, whereas internal organ manifestations and the frequency of autoantibodies are far less pronounced than in adult-onset SSc. Also, the survival rate and final outcome of patients with jSSc appear to be better than those in patients with adult-onset SSc. Ann. N.Y. Acad. Sci. 1051: 229–234 (2005). doi: 10.1196/annals 1361.063. (Also see: Dr. László Czirják) |
| Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. The insidious onset of the disease, the delay in diagnosis, the recognition of mixed subtype and the better definition of the other subtypes should influence our efforts in educating trainees and practitioners and help in developing a comprehensive classification system for this syndrome. PubMed. Rheumatology (Oxford). 2005 Dec 20. (Also see: Localized Scleroderma) |
| Comparison of Clinical and Serologic Findings in Childhood and Adult Onset Systemic Sclerosis. Childhood onset SSc is similar to adult onset disease with the exception that ChO patients more frequently have overlap syndromes, skeletal muscle involvement and anti-PM-Scl and anti-U1 RNP antibodies. ChO patients less frequently have anticentromere and anti-RNA polymerase III antibodies and renal crisis. Thaschawee Arkachaisri. 1838. ACR 2004. (Also see: Types of Scleroderma ) |
| 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. |
| Aspects of nailfold capillaroscopy in children and young people with rheumatic disease. Distinctive morphologic abnormalities with capillary enlargement, megacapillary and dropout of surrounding structures were noted in two groups: patients with childhood dermatomyositis (DM) and with scleroderma (SD). Capillary abnormalities were found in all 4 patients affected by systemic disease and in none of 7 patients with localized scleroderma. PubMed. Rom J Intern Med. 2004;42(1):211-5. |
| Autoantibodies to Beta Adrenoreceptors in Patients with Juvenile Scleroderma. Further follow up investigations are necessary to find out whether ABâ-1 could serve as an early predictor of scleroderma cardiac lesions in the pre-clinical stage of the disease. M. K. Osminina. THU0095 EULAR 2004. (Also see: Antibodies and Cardiac Involvement) |
| Paediatric idiopathic inflammatory muscle disease. JDM (juvenile dermatomyositis) has a significantly different spectrum of disease from adult dermatomyositis. Juvenile myositis can also occur as part of other systemic autoimmune diseases such as scleroderma and systemic lupus erythematosus. PubMed. Best Pract Res Clin Rheumatol. 2004 Jun;18(3):345-58. (Also see: Types of Scleroderma and Dermatomyositis) |
| Raynaud's phenomenon in children. It is important to distinguish primary Raynaud's phenomenon, which is usually benign, from Raynaud's phenomenon secondary to connective tissue disease. PubMed. Arch Pediatr. 2004 Jan;11(1):74-7. (Also see: Raynaud's) |
| Serum Level of KL-6 as a Marker of Interstitial Lung Disease in Patients with Juvenile Systemic Sclerosis. Its advantages over conventional methods of ILD assessment, such as pulmonary function test and high-resolution computerized tomography, are that it is easy to quantify and to measure repeatedly and it does not need children's cooperation. J Rheumatol. April 2004;31:795-800. |
| Systemic Scleroderma Syndromes (SSS) in Children: Clinical and Immunological Characteristics of 181 Patients. This comprehensive data collection represents the starting point for the definition and validation of diagnostic and classification criteria of juvenile SSS. Giorgia Martini. ACR Conference Oct. 2003. |
| Juvenile Localized Scleroderma: Clinical Epidemiological Features of 688 Patients. This represents the largest data collection of children with JLS and the starting point for a better definition of the diagnostic criteria and for the validation of the classification in use. Francesco Zulian. ACR Conference Oct. 2003. |
| Cyclophosphamide pulse therapy for pediatric systemic sclerosis. We encountered three patients with pediatric systemic sclerosis. It became possible for patient 1 and 2 to achieve and maintain a marked improvement of the clinical manifestations as a result of cyclophosphamide pulse therapy early in the course of the disease. PubMed. Ryumachi. 2003 Oct;43(4):660-6. |
| Immunological Features and Nail-Fold Capillary Patterns in 32 Cases of Raynaud's Phenomenon in Childhood. The Raynaud's phenomenon (RP) is thought to occur rarely in childhood, and when it does occur, it is usually due to an underlying connective tissue disease. RP in childhood prevailed in girls with a mean age 14.5 years, with the diagnostic of CTD in 31.2% of the patients. In the patients with RP and positive ANA, CTD/scleroderma pattern was observed, confirming the CTD diagnosis.J. Bethencourt. AB0472 EULAR 2003 (Also see: Raynaud's) |
| Elevated Levels of Serum N-Acetyl-Beta-D-Hexoaaminidase (NAHASE) Activity in Childhood Scleroderma. As previously described in systemic sclerosis of adult pts, in children, serum NAHase activity was elevated not only in systemic sclerosis but in localized scleroderma as well. Our results suggest that alteration in serum NAHase activity may be closely related to the atrophic process in LS.E. Musiej-Nowakowska. FRI0089 EULAR 2003 (Also see: Types of Scleroderma) |
| Safety and efficacy of iloprost for the treatment of ischaemic digits in paediatric connective tissue diseases. Iloprost appears to be a safe and effective treatment for ischaemic digits and digital ulcers in children with CTD. In conjunction with immunosuppressive drugs, it has a potential role in preventing irreversible complications, such as digital gangrene and amputationPubMed. Rheumatology (Oxford). 2003 Aug 15 (Also see: Digital Ulcers) |
| A case of juvenile systemic sclerosis with disease onset at six months old. The occurrence of SSc in childhood is rare, with childhood-onset disease representing 3% of all SSc cases, and fewer than 100 such patients have been reported in the world literature to date.PubMed. Clin Rheumatol 2003 May;22(2): 162-3 |
| Raynaud's phenomenon in children: a retrospective review of 123 patients. RP in children, as in adults, principally affects girls and is frequently free of association with connective tissue disease. Antinuclear antibody positivity and abnormal nailfold capillaries correlate with secondary disease. Antiphospholipid antibodies are surprisingly common, a new finding of uncertain implications.PubMed. Pediatrics 2003 Apr;111(4 Pt 1):715-21 (Also see: Raynaud's) |
| Nailfold capillary microscopy in healthy children and in childhood rheumatic diseases: a prospective single blind observational study. This study is unique in measuring objective quantitative and qualitative parameters of the nailfold vasculature across a wide spectrum of age and disease. Differences in capillary morphology and frequency in children with CTD compared with other paediatric diseases and healthy controls were demonstrated.PubMed. Ann Rheum Dis 2003 May;62(5):444-9 |
| Phototherapy in pediatric patients. The treatment of children with psoriasis, atopic dermatitis (AD), pityriasis lichenoides, and scleroderma poses a therapeutic problem because all therapeutic options are associated with numerous side effects. We conclude that phototherapy and PUVA bath are valuable and safe therapeutic options for selected children who do not respond to other treatments. PubMed. Pediatr Dermatol 2003 Jan-Feb;20(1):71-7 |
| Severe Cardiac Involvement in Children with Systemic Sclerosis and Myositis Children with diffuse cutaneous SSc and features of polymyositis are prone to develop severe cardiomyopathy. Combination therapy of corticosteroids, MTX, and cyclosporine seems to be active on muscle, skin, and lung involvement but does not impair progression of esophageal or myocardial dysfunction. The Journal of Rheumatology VOLUME 29: NO. 8 AUGUST 2002 |
| Linear Scleroderma in children PUBMED Abstract |
| Scleroderma Support Groups ISN |
| Patient Stories |
| Morphea Stories |
| Linear Stories |
| Allen's Mom: Son has Scleroderma With his tightness of skin he has a very hard time bending, walking up the stairs and for the most part he cannot walk for more than a few minutes without his legs giving out... |
| Anita G: Localized Scleroderma This also started as a small spot that I thought was insignificant, but as time went on, it grew, along with my symptoms... |
| Chris S: Mother of Linear Morphea Patient Whatever it was, I knew it should not be there... |
| Deedra: Linear Scleroderma In the course of two years the scleroderma spread from under my knee to the top of my thigh... |
| 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... |
| Emma C: Living with Localized Morphea Some of my earliest childhood memories are of my mother taking me to see doctors who were baffled by the strange marks on my back... |
| 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... |
| Federica: Morphea I have had localized morphea in my lower back for two years... |
| Georgina: Mother of Child with Morphea The only thing I know about it is what her doctor told me, namely that it has no cure... |
| Georgina: Madre de Paciente con Morfea Lo único que sé es que es una enfermedad incurable, por que es lo único que me explico su doctor... |
| Ginger: Generalized/Linear Scleroderma After analyzing all the test results and my medical records the neurologist attributed the muscle atrophy to the linear scleroderma... |
| Hope: Linear Morphea My mom took me to the doctor and for three months he treated it as ringworm... |
| 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"... |
| 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... |
| Karina: Mother of Morphea Patient The doctor says it will be under control, and that it is a good thing that it is localized and hasn't spread... |
| Laura H: Mother of Son with Linear Morphea What started as a bruise in his forehead, has come down to his nose, and his forehead has sunk a bit... |
| (Español/Spanish) Laura H.: Morfea Lineal Al principio no le dí mucha importancia porque pasaba el tiempo y seguía igual, pero ahora pasado un año todo ha cambiado... |
| Lindsey Z: Morphea Scleroderma I was self conscious in fear that others would care, or find me ugly because of it... |
| 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... |
| Mary L: Morphea My mom had the department of child services call on
her because someone said I was all "bruised".
I was denied enlistment into the United States Army because of this disease... |
| 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... |
| Tori: Localized Juvenile Scleroderma This is not the first rare disease that I have had... |
| Tyler: Scleroderma, CREST Syndrome It is really confusing and the doctors say it is very rare for people my age to have it... |
| Yally: Dermatitis with Linear/Morphea My daughter has a spot on her left leg, that almost covers it completely... |
| Patient & Caregiver Stories (Main Collection) ISN |
