[Skip to Content]
Navigation
Home > Medical Directory > What is Scleroderma? > Types of Scleroderma > Systemic > Symptoms > Skin

Skin Fibrosis

This initial page was excerpted with permission from "Understanding Scleroderma" by Dolores Vazquez-Abad, M.D. Copyright © 1997. Items have been added to this page since then, as noted by dates. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
What is Fibrosis?
Diagnosis
Progression
Physical Therapy
Oral Hygiene
Treatments
d-Penicillamine
Spontaneous Remission
Myositis (Muscle Inflammation)
Research
Personal Stories of Skin Fibrosis

What is Fibrosis?

Fibrosis is a process that follows chronic inflammation. Fibrotic tissue is like a scar tissue, thick, and rigid, due to excess accumulation of protein below the skin.
Molecular pathogenesis of skin fibrosis: insight from animal models. Skin fibrosis occurs in a variety of human diseases, most notably systemic sclerosis (SSc). The end stage of scleroderma in human skin consists of excess collagen deposition in the dermis with loss of adnexal structures and associated adipose tissue. Smith GP. (PubMed) Curr Rheumatol Rep. 2010 Feb;12(1):26-33.

Diagnosis of Skin Fibrosis

Scleroderma is named after skin fibrosis, which is one of its most common and recognizable symptoms. Skin fibrosis eventually develops in most patients. Sclero means "hard" and derma means "skin". The diagnosis of skin fibrosis is clinical and it requires no laboratory or special testing.
Doctors may request skin biopsies when there are unusual patterns or areas of skin that become tight and firm. (Also see Types of Scleroderma)
Assessment of skin microcirculation by laser Doppler flowmetry (LDF) in systemic sclerosis patients (SSc). LDF is suitable for the assessment of the microangiopathy degree in SSc patients. Ter Media, Postep Derm Alergol, 03/05/2014.
Virtual skin biopsy by optical coherence tomography: the first quantitative imaging biomarker for scleroderma. The longitudinal study to test the sensitivity of the OCT based algorithm will tell whether this can used to determine changes in skin fibrosis over time and therefore used as an outcome measure in clinical trials and in clinical management. PubMed. 02/20/2013.

Progression

The usual presentation is distal to proximal: finger tips first with progression to the fingers, hands, forearms, and arms. By the time the skin of the arms is tight, there may be stiffness of the legs, thighs, and in some cases, chest and abdomen. The pattern of skin stiffness is usually bilateral and symmetrical.
The skin of the face and neck may be involved in the mild, also called localized Scleroderma (with only finger tightness), or in proximal or diffuse Scleroderma (tight skin proximal to the hands).
Biomarkers for skin involvement and fibrotic activity in scleroderma. This review summarizes all non-invasive physical and laboratory examinations, which permit a better understanding of the fibrotic activity of the disease, can be effectively used to assess potential therapeutic response and help to find better treatment options. Moinzadeh P. (PubMed) J Eur Acad Dermatol Venereol. 2011 Aug 8. (Also see: Biomarkers)
Progressive Loss of Lymphatic Vessels in Skin of Patients with Systemic Sclerosis (SSc). In SSc, lymphatic microangiopathy is linked to the progression of skin involvement. The progressive disappearance of lymphatic vessels may have a critical pathogenetic role in the progression of SSc from an early edematous phase to overt fibrosis. Mirko Manetti. The Journal of Rheumatology February 1, 2011 vol. 38 no. 2 297-301.
Spontaneous skin regression and predictors of skin regression in Thai scleroderma patients A spontaneous regression of the skin fibrosis process was not uncommon among Thai SSc patients. The factors suggesting a poor predictor for cutaneous manifestation were Raynaud's phenomenon, diffuse cutaneous type while early cyclophosphamide therapy might be related to a better skin outcome. Chingching Foocharoen, Clinical Rheumatology, March 24, 2011.

Physical Therapy

At the first stages of skin tightening on the fingers, hands, and legs occupational and physical therapy are important in preventing, and ameliorating irreversible limiting contractures of the fingers.
Protection of the hand, and fingers by utilization of gloves when doing the dishes, gardening, and other physical activities prevents trauma that may break the skin, and produce slow healing ulcers with risk of infection.

Oral Hygiene

Oral hygiene may become difficult in cases where facial skin becomes tight. You must make frequent appointments with your dentist, who should be aware of your disease and maintain communication with your doctor. (Also see Dental Involvement)

Treatments of Skin Fibrosis

Low dose UVA1 phototherapy has been found especially effective for morphea and also, in some cases, for systemic sclerosis.
When the skin tightening progresses quickly, or involves the chest and abdomen, your doctor may choose among the medications currently used for this condition.
Treating skin and lung fibrosis in systemic sclerosis: a future filled with promise? The most promising targets include inhibitors of B-cells, tyrosine kinases, 5-hydroxytryptamin receptors, interleukin-6 and Wnt signalling. PubMed, Curr Opin Pharmacol, 2013 Jun;13(3):455-62. (Also see: Pulmonary Fibrosis Research)
Low-dose UVA1 phototherapy for scleroderma: what benefit can we expect? In patients with morphea, a marked improvement was found in 77.8% patients and a moderate improvement was found in 11.1% patients. In the systemic scleroderma group, a patient with complete remission of the skin sclerosis is emphasized. PubMed. J Eur Acad Dermatol Venereol. 2012 May;26(5):619-26. (Also see: Morphea Treatments)
UVA1 Phototherapy: A Concise and Practical Review. Phototherapy is an effective therapeutic option in scleroderma and should be considered among the first approaches in the management of localized scleroderma or morphea. UVA1 phototherapy has also been used for patients with limited and diffuse systemic sclerosis. SkinTherapyLetter, 06/21/12. (Also see: Morphea Treatments)
Treatment of Systemic Sclerosis Complications: What to Use When First-Line Treatment Fails—A Consensus of Systemic Sclerosis Experts. Symptoms treatment recommendations by over 100 scleroderma experts, for scleroderma renal crisis (SRC), digital ulcers (DU), pulmonary hypertension (PAH/PH), reflux, skin involvement, and arthritis. Seminars in Arthritis and Rheumatism Volume 42, Issue 1 , Pages 42-55, August 2012. (Also see: Scleroderma Renal Crisis, Digital Ulcers, Pulmonary Hypertension, Reflux, Skeletal Involvement, Scleroderma Treatments, and Dr. Janet Pope)

Penicillamine

A multicenter double blinded study in 1997 showed that d-Penicillamine does not soften the skin, and that there was no improvement in internal organ involvement from d-Penicillamine. (Also see Clinical Trials: Ineffective or Unproven Treatments)
Cat by Lisa Volz, ISN ArtistA word of caution regarding single-center, retrospective studies. Patients frequently improve on placebo. Patients who are improving usually attribute it to their therapy and thus stay with it. Thus uncontrolled cohorts become enriched for "responders" while failures seek other paths. This dynamic underlies virtually all therapeutic "breakthroughs" which is why large scale double-blinded clinical trials are crucial for determining valid scleroderma treatments.

Methotrexate

At present a similar study is being conducted to evaluate the role of Methotrexate. (1) (Also see Clinical Trial: Open Enrollments)

Spontaneous Remission

In addition, anecdotal reports of spontaneous remission are well known. Consequently, the initiation of specific therapy for this condition should be carefully evaluated and followed by your doctor. If started, all of these drugs require frequent laboratory monitoring for toxicity, and secondary effects. (1)

Myositis (Muscle Inflammation)

The skin tightening may occur rapidly, producing sudden increase in the pressure under the skin, and rubbing of the muscles and tendons below the skin. This may cause inflammatory muscle disease (Myositis), which frequently accompanies Scleroderma. In these cases, a blood test will show elevation of muscle enzymes.
Cessation of exercise, especially isotonic exercises that require repeated contraction of muscle groups, is mandatory. Isotonic exercises will increase the rubbing produced by the thickened skin, and augment the inflammation in the muscles. Your doctor may contemplate the use of steroids according to the degree of myositis. Repeated blood work for detection of muscles enzymes should be performed at your doctor's discretion. (Also see Skeletal Involvement)

Research

What does global gene expression profiling tell us about the pathogenesis of systemic sclerosis? Global gene expression profiling in skin and peripheral blood can contribute to a better understanding of SSc pathogenesis and identify novel biomarkers and therapeutic targets. PubMed, Curr Opin Rheumatol, 2013 Nov;25(6):686-91.
Preclinical and translational research to discover potentially effective antifibrotic therapies in systemic sclerosis (SSc). There is a high unmet clinical need for effective antifibrotic therapies in systemic sclerosis (SSc), and in parallel a rapid development in the identification of potential molecular targets in preclinical research. This could increase the possibility to develop successful drugs against the fibrotic manifestations of SSc. PubMed, Curr Opin Rheumatol. 2013 Nov;25(6):679-85. (Also see: Scleroderma Clinical Trials)
Scientists identify agent that can block fibrosis of skin, lungs. Researchers at the University of Pittsburgh School of Medicine have identified an agent, E4 (which is a piece of protein or peptide derived from endostatin) that in lab tests protected the skin and lungs from fibrosis. MedicalXpress. 05/30/12. (Also see:Pulmonary Fibrosis Treatments)
Enhanced expression of ephrins and thrombospondins in the dermis of patients with early diffuse systemic sclerosis (SSc): potential contribution to perturbed angiogenesis and fibrosis EphB4 and EphrinB2 are up-regulated in clinically involved skin of SSc patients, suggesting their participation in SSc-perturbed angiogenesis. TSP1 and TSP2 are up-regulated in both clinically involved and non-involved SSc skin and are constitutively overexpressed in a TGF-β-dependent and hypoxia-independent manner in SSc dermal fibroblasts, suggesting their potential early contribution in SSc pathogenesis. Avouac J, Rheumatology, March 30, 2011.

Personal Stories of Skin Fibrosis

Alana: Scleroderma (Peru) I cannot open my mouth completely, I have lost strength in my hands, I have breathing problems, my face looks different, my skin is really tight and I have lost my appetite...
(Español/Spanish) Alana: Paciente Nuevo con Esclerodermia (Peru) Le dije que ya no podía abrir la boca completamente, he perdido la fuerza de las manos, tengo problemas respiratorios, mi rosto está diferente, mi piel está dura y he perdido casi el apetito...
Alexandra: Scleroderma My scleroderma symptoms arrived in great style: reflux, itchy swollen skin, joint and muscle pain and tiredness. Plus hundreds of little red spots (telangiectasia). My mouth was tight and getting smaller too...
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...
Dawn M: Linear/Systemic Scleroderma My family and I were informed by the doctors, that the localized/linear form of scleroderma that I was diagnosed with, would never progress into the potentially fatal, systemic form...
Gioia: Systemic Progressive Sclerosis (Italy) I was unable to swallow food, and a few times I was at risk of suffocating, and I also had terrible ulcers in my hands...
(Italiano) Gioia: Sclerosi Sistemica Progressiva (Italia) Poi però mi sono resa conto a mie spese che non era così, non riuscivo più a deglutire il cibo, più di qualche volta ho rischiato di soffocarmi, per non parlare delle ulcere alle mani...
Heather A: Scleroderma (South Africa) My fingers started to curl up and I could not wear any rings on my fingers anymore as they were so swollen...
Kamlesh: Husband of Limited Scleroderma Patient (India) In a pulmonary function test, there was severe restriction and severe obstruction...
Karligash: Systemic Scleroderma (Republic of Kazakhstan) Young, beautiful, full of hope and expectations for my life, for happiness and love — that was me, nineteen years of age...
(Russian) Карлыгаш: системная склеродермия (Республика Казахстан) Молодая,красивая,полная ожидания от жизни счастья,любви такая я была в 19 лет...
Kristi U: Systemic Scleroderma We sold our motel business because it hurt me so bad to work each and every day...
Laira: MCTD, Scleroderma, SLE, Lupus, CREST, Lymphoma I was just seventeen when I was told I had rheumatoid arthritis...
Leslie R: Scleroderma, Vitiligo, Lupus, Anemia, Hypertension and Type 2 Diabetes He told me that I have scleroderma and explained what this disease is about. After suffering so long I finally got some answers...
Manu: Daughter of Patient with Systemic Scleroderma (Italy) I recently lost my father, and in his last moments he suffered very much. He was affected by systemic scleroderma that began with simple inflammation of his hand in November of 2006...
(Italiano) Manu: Figlia di Padre Malato di Sclerodermia Sistemica (Italia) Ho perso mio papà una settimana fa, dopo atroci sofferenze. Era affetto da sclerodermia sistemica iniziata con un banale gonfiore di una mano nel novembre 2006...
Matilda: Systemic Scleroderma (South Africa) I don't think there is a lot of support in South Africa and I would actually like to start a group where people with the same disease can meet and discuss how they cope with the illness...
Sheila Z: Scleroderma I would like to first say that this site has been a great help for me to understand what is happening to my body and my life...
Sibel: Scleroderma and Raynaud's (Turkey) Changes in my skin were making me look different in the mirror...
Sue D: Diffuse Scleroderma Pain developed in my hands, then I noticed pain in my knees, then my shoulders, down my back, elbows, hips, feet...
Susan L: Diffuse Scleroderma I first noticed the swelling in my hands and feet shortly after my daughter was born in 2005, and thought that it was post-pregnancy fluid...
Theon: Scleroderma and Pneumothorax This is very hard for me, because I was a very active woman, and then suddenly I am totally and completely disabled...
 
Go to Gangrene/Amputation
About the ISN

This website is certified by Health On the Net Foundation. Click to verify.We are a full-service nonprofit 501(c)(3) providing stellar worldwide research, support, education and awareness for scleroderma and related illnesses, such as pulmonary hypertension. This site complies with the HONcode standard for trustworthy health information: verify here.

 

Scleroderma Hotline (English Only)
Toll Free U.S. Call 1-800-564-7099
Direct Line: Call U.S. 952-831-3091

Post a message in Sclero Forums
Email: isn@sclero.org

International Scleroderma Network (ISN)
7455 France Ave So #266
Edina, MN 55435-4702
United States

AKA Scleroderma from A to Z and SCLERO.ORG
Privacy Policy, Financial Disclosure, and Disclaimer.

Home   Medical   News   Sclero Forums   Support   Translations

© Copyright 1998-2014 International Scleroderma Network
All Rights Reserved