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Scleroderma: Who gets and causes


Who gets scleroderma?

Scleroderma is rare. The Scleroderma Foundation estimates that about 300,000 people in the United States have some type of scleroderma.

The following explains who has a higher risk of developing scleroderma.

Gender: Women are more likely than men to get most types of scleroderma. One type, linear morphea, occurs about equally in men and women.

Age: This disease can occur at any age. Children and seniors get scleroderma. One type, linear morphea, tends to develop before 18 years of age. The other types are most likely to begin between the ages of 30 and 50.

Race: Whites and Asians are most likely to get the types of scleroderma that affect only the skin and the tissue that lies beneath.

The Choctaw Native Americans in southeastern Oklahoma seem to have the highest risk of developing scleroderma that affects the skin and internal organs.

Several studies have shown that African Americans also have a higher risk of developing scleroderma that affects the skin and internal organs. In the United States, about 70% of people who get this type are African American.

What causes scleroderma?

Researchers are looking into what actually causes scleroderma. We do know that this group of rare diseases is:

  • NOT contagious

  • NOT a type of cancer

  • NOT an infection

We also know that a person develops scleroderma when the body makes too much collagen. This excess collagen is what causes the skin to thicken and harden. It can also cause hardening and thickening in the tendons, joints, and parts of the internal organs.

Why this happens is still a bit of a mystery. It’s believed that something in the person’s immune system goes wrong, causing the body to make too much collagen.

It’s also possible that something in the person’s environment could trigger scleroderma.

As far back as 1914, we’ve noticed that scleroderma is more common in people exposed to silica dust. Those exposed to silica dust on the job include miners, foundry workers, and roofers. More research is needed, however, to know whether silica dust increases the risk.

We also know that children who develop morphea, the most common type of scleroderma, are more likely to have a blood relative who has morphea. Genes may play a role.


References
Denton, CP and Black CM. “Scleroderma (systemic sclerosis).” In: Wolff K, Goldsmith LA, et al. Fitzpatrick’s Dermatology in General Medicine (seventh edition). McGraw Hill Medical, New York, 2008:1553-62.

Falanga V and Killoran CE. “Morphea.” In: Wolff K, Goldsmith LA, et al. Fitzpatrick’s Dermatology in General Medicine (seventh edition). McGraw Hill Medical, New York, 2008:543-6.

Fett N and Werth VP. “Update on morphea Part I. Epidemiology, clinical presentation, and pathogenesis.” J Am Acad Dermatol 2011;64(2):217-28.

Fett N and Werth VP. “Update on morphea: Part II. Outcome measures and treatment. J Am Acad Dermatol 2011;64(2):231-42.

Marie I, Menard JF, et al. “Association of occupational exposure with features of systemic sclerosis.” J Am Acad Dermatol. 2015;72(3):456-64.

Martin JR, Griffin M, “Systemic sclerosis (scleroderma) in two iron ore mines.” Occup. Mod. 1999;49:161-9.

McKinley-Grant L, Warnick M, et al. “Cutaneous manifestations of systemic disease.” In: Kelly AP and Taylor SC. Dermatology for skin of color. McGraw-Hill Companies, China, 2009:495-7.

Rőcken M and Ghoreschi K. “Morphea and lichen sclerosus.” In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:1469-76.

Scleroderma Foundation. “What is scleroderma?” Website last accessed February 2016.

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