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Scleroderma: Types, Causes, Symptoms, Diagnosis, Treatment And Management

Scleroderma or systemic sclerosis (SSc) or CREST syndrome is a rare autoimmune disorder recognised by fibrosis (scar tissues), inflammation, tightening, and hardening of the skin and other internal organs like the digestive tract, blood vessels, heart, kidney, bones and muscles. It is caused due to the malfunction of the body's immune system which attacks its own organs and tissues. [1]

The exact cause of scleroderma is still unknown. However, it is mainly caused due to excess production of collagen, a protein found in the bones, skin, muscles and tendons of mammals which are responsible to provide strength and structure to the body. Collagen is also an important component of connective tissues in our body.

Scleroderma affects 1 out of every 100000 individuals. Mostly it is found in Australia, the USA and Eastern Europe. Also, it occurs 4 times more in women than men between the age of 30-50. Scleroderma is a chronic disease like diabetes which cannot be treated but, only the symptoms are managed. [2]

Types Of Scleroderma

There are 2 main types of scleroderma.

1. Systemic sclerosis: It affects the whole body including major organs and blood circulation system. SSc is again divided into two parts which are as follows: [3]

  • Limited cutaneous systemic sclerosis (LCSS): Also known as CREST syndrome, LCSS affects a particular body area like the skin of hands, legs, fingers and face. It is also known as CREST syndrome as the symptoms denote calcinosis (deposition of calcium under the skin), Raynaud phenomenon (interruption in blood flow), oesophageal dysfunction (difficulty in swallowing), sclerodactyly (inward curl of fingers and telangiectasia (small red spots on hands and face). [4]
  • Diffuse cutaneous systemic sclerosis: It is life-threatening and progressive. They affect major skin areas, internal organs like heart, lungs and kidney and also cause joint problems.

2. Localised scleroderma: It does not cause any harm to the internal organs and referred to as the mildest form of scleroderma. It is characterised by waxy patches, hard and thick skin which is red or white along with purple borders. [5]

Causes Of Scleroderma

As aforementioned, scleroderma is caused due to the excess production of collagen that holds the body tissues together. Researchers say that there's no exact cause for the overproduction of collagen in the body. However, they believe that such conditions are not hereditary but usually affect individuals who have other autoimmune conditions in their families like lupus and rheumatoid arthritis. [1]

The other cause which may lead to scleroderma is environmental factors. People who are more exposed to silica or certain varieties or drugs are at risk of developing this rare condition. Such people develop scleroderma due to variation in their genes for some unknown conditions. Basically, the gene variation is the result of both genetic and environmental factors. [6]

Symptoms Of Scleroderma

The symptoms of scleroderma are as follows:

  • Puffiness of hands and feet [1]
  • Calcinosis
  • Stiffness in the joints
  • Pain in the joints
  • Red spots on the skin
  • Shinny skin due to tightening [7]
  • Heartburn due to acid reflux
  • Fatigue
  • Persistent cough
  • Bloating
  • Dry eyes (Sjögren's syndrome)
  • Diarrhoea or constipation [8]

Risk Factors Of Scleroderma

Though scleroderma is rare, it can happen to any individual. Following are the risk factors which may lead a person to this condition:

  • Genes: Though the condition is not genetic, yet there are several pieces of evidence which shows that people of Native American (Choctaw), African-Americans and European ancestry are more prone to the disorder. [9]
  • Environment: High exposure to silica dust, certain chemotherapy drugs (Bleomycin) and organic solvents makes people at high risk of the disease. [6]
  • Autoimmune disease: If there's a previous history of an autoimmune disorder (like lupus, Guillain-Barre syndrome and inflammatory bowel disease) in the family, there's a higher risk of transfer of the gene to the other family members. [10]
  • Gender: The female to male ratio in scleroderma is 3:1 as the onset of the condition is earlier in female compared to male. [2]

Complications Of Scleroderma

Complications of scleroderma include the following:

  • Scarring of the lung tissues
  • Abnormal production of saliva
  • Rapid kidney failure [11]
  • Skin sores on the fingertips
  • Congestive heart failure
  • Heart palpitation
  • Erectile dysfunction
  • Loss of libido in women [12]
  • Heartburn, diarrhoea and other digestive problems

Diagnosis Of Scleroderma

Scleroderma appears in many forms which makes it hard to diagnose. However, it is carried out by the following tests:

  • Skin biopsy to rule out other skin disorders [13]
  • Genetic testing to get detailed knowledge of the genes of the patient
  • Blood tests to examine the levels of particular antibodies
  • Microscopic examination to check for changes in blood capillaries in the skin near fingernails
  • The medical expert will also perform several tests to identify symptoms like internal organ damage and skin thickening.

Treatment Of Scleroderma

Some treatment methods for scleroderma is very effective to manage its symptoms. However, there is no particular drug which totally reverses or treats the condition. The common treatment methods for scleroderma include the following:

1. Medications: Include the drugs to control the symptoms of scleroderma [14]

  • Angiotensin-converting enzyme inhibitors to prevent kidney damage
  • PDE-5 inhibitors to prevent Raynaud's phenomenon
  • Antacid drugs to prevent GERD and acid reflux
  • Anti-inflammatory drugs to prevent muscle weakness and joint dysfunction
  • Steroid creams to reduce the stiffness of the skin
  • Immune system suppressant to reduce the effect of the immune system on the body.
  • Antibiotics to prevent cold and other infections

2. Surgery: The surgical process include the following:

  • Lung transplant: In the case of serious lung damage by scleroderma [15]
  • Amputation: In the case of Raynaud's disease

How To Manage Scleroderma

  • Take good care of your skin
  • Protect your skin from the sun regularly
  • Avoid using harsh or chemical-based soaps
  • Avoid hot bath or exposure to very cold temperature
  • Quit smoking [16]
  • Avoid late-night foods to prevent heartburn
  • Do appropriate exercise to prevent muscle stiffness
  • Moisturize your skin all the time
  • Manage your stress and anxiety by performing yoga or meditation
  • Prevent the intake of excess sugar or fried foods
View Article References
  1. [1] Badri T, Hariz A. Scleroderma. [Updated 2019 Jan 20]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-.
  2. [2] Peoples, C., Medsger, T. A., Jr, Lucas, M., Rosario, B. L., & Feghali-Bostwick, C. A. (2016). Gender differences in systemic sclerosis: relationship to clinical features, serologic status and outcomes. Journal of scleroderma and related disorders, 1(2), 177–240. doi:10.5301/jsrd.5000209
  3. [3] Viswanath, V., Phiske, M. M., & Gopalani, V. V. (2013). Systemic sclerosis: current concepts in pathogenesis and therapeutic aspects of dermatological manifestations. Indian journal of dermatology, 58(4), 255–268. doi:10.4103/0019-5154.113930
  4. [4] digun R, Hariz A. Systemic Sclerosis (CREST syndrome) [Updated 2019 Jan 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-.
  5. [5] Careta, M. F., & Romiti, R. (2015). Localized scleroderma: clinical spectrum and therapeutic update. Anais brasileiros de dermatologia, 90(1), 62–73. doi:10.1590/abd1806-4841.20152890
  6. [6] Mora, G. F. (2009). Systemic sclerosis: environmental factors. The Journal of rheumatology, 36(11), 2383-2396.
  7. [7] Anbiaee, N., & Tafakhori, Z. (2011). Early diagnosis of progressive systemic sclerosis (scleroderma) from a panoramic view: report of three cases. Dento maxillo facial radiology, 40(7), 457–462. doi:10.1259/dmfr/64340754
  8. [8] Shreiner, A. B., Murray, C., Denton, C., & Khanna, D. (2016). Gastrointestinal Manifestations of Systemic Sclerosis. Journal of scleroderma and related disorders, 1(3), 247–256. doi:10.5301/jsrd.5000214
  9. [9] Mayes M. D. (2012). The genetics of scleroderma: looking into the postgenomic era. Current opinion in rheumatology, 24(6), 677–684. doi:10.1097/BOR.0b013e328358575b
  10. [10] Cárdenas-Roldán, J., Rojas-Villarraga, A., & Anaya, J. M. (2013). How do autoimmune diseases cluster in families? A systematic review and meta-analysis. BMC medicine, 11, 73. doi:10.1186/1741-7015-11-73
  11. [11] Mouthon, L., Bérezné, A., Bussone, G., Noël, L. H., Villiger, P. M., & Guillevin, L. (2011). Scleroderma renal crisis: a rare but severe complication of systemic sclerosis. Clinical reviews in allergy & immunology, 40(2), 84-91.
  12. [12] Frikha, F., Masmoudi, J., Saidi, N., & Bahloul, Z. (2014). Sexual dysfunction in married women with Systemic Sclerosis. The Pan African medical journal, 17, 82. doi:10.11604/pamj.2014.17.82.3833
  13. [13] Busquets, J., Del Galdo, F., Kissin, E. Y., & Jimenez, S. A. (2010). Assessment of tissue fibrosis in skin biopsies from patients with systemic sclerosis employing confocal laser scanning microscopy: an objective outcome measure for clinical trials?. Rheumatology (Oxford, England), 49(6), 1069–1075. doi:10.1093/rheumatology/keq024
  14. [14] Frech, T. M., Shanmugam, V. K., Shah, A. A., Assassi, S., Gordon, J. K., Hant, F. N., … Domsic, R. T. (2013). Treatment of early diffuse systemic sclerosis skin disease. Clinical and experimental rheumatology, 31(2 Suppl 76), 166–171.
  15. [15] Crespo, M. M., Bermudez, C. A., Dew, M. A., Johnson, B. A., George, M. P., Bhama, J., ... & Pilewski, J. M. (2016). Lung transplant in patients with scleroderma compared with pulmonary fibrosis. Short-and long-term outcomes. Annals of the American Thoracic Society, 13(6), 784-792.
  16. [16] Leask A. (2011). When there's smoke there's…scleroderma: evidence that patients with scleroderma should stop smoking. Journal of cell communication and signaling, 5(1), 67–68. doi:10.1007/s12079-010-0111-1

Story first published: Monday, December 2, 2019, 8:00 [IST]
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