Scleroderma: Myths of the Rare Disease

Information About Scleroderma 

Scleroderma has been sectioned into two forms: systemic sclerosis and localized scleroderma. Systemic sclerosis (SSc) is a chronic autoimmune disease. Its most prominent feature is the process of progressive fibrosis resulting from the excessive deposition of extracellular matrix components in different tissues and organs. Systemic sclerosis usually affects the internal organs or internal systems of the body, such as the lungs, heart, kidneys, musculoskeletal system, and gastrointestinal tract. Localized sclerosis, also known as morphed or linear, is an autoimmune condition that affects a local area of skin, causing the formation of one or more patches of inflammation of the skin and muscles. This inflammation triggers connective tissue cells to produce too much collagen causing subsequent hardening. It also affects tissue such as muscle and bone under the skin. 

The symptoms of scleroderma, particularly systemic sclerosis, include bloating after eating, diarrhea, and an alternation of diarrhea and constipation. Joint mobility of patients is reduced as there might be stiffening and swelling of fingers and joints. 

Some factors, such as gender, environment, and genes, may be the triggering causes of scleroderma. Women and people with family members who have autoimmune diseases, such as lupus, are more likely to get scleroderma. Silica exposure from coal mining or sandblasting increases the risk of systemic scleroderma. 

Additional Conditions Regarding Scleroderma 

One condition that is closely related to scleroderma is drug-induced scleroderma-like lesions. 

This is a condition in which the administration of a drug induces skin sclerotic lesions similar to either systemic or localized sclerosis. Drug-induced scleroderma-like lesions can be caused by a large variety of drugs such as bleomycin, L-tryptophan, vinyl chloride, and phytonadione (vitamin K1). However, in recent years, the number of cases caused by these drugs has decreased. 

Another condition that is closely related to scleroderma is Raynaud’s phenomenon. This affects the body’s ability to supply blood to certain parts of the body, usually triggered by cold temperatures, anxiety, or stress. It is commonly associated with scleroderma as it is triggered by an inappropriate and exaggerated contraction of the small blood vessels in the fingers and toes. When this occurs, the affected area may change color to white, then blue, with the onset of numbness, pain, and pins and needles in the area thereafter. 

Myths About Scleroderma

Some of the most common misconceptions about scleroderma are as follows:

1. Scleroderma is contagious.

2. Patients who have been infected with scleroderma only have 5 years left to live.

3. Only the skin is affected by scleroderma.

4. Only adults can have scleroderma. 

5. Scleroderma is a genetic disease.

6. Scleroderma is curable.

The last misconception is extremely unfortunate. Currently, there are treatments and medications to help manage the symptoms of the disease, such as prednisone, sumatriptan, famotidine, escitalopram, and tramadol. However, scleroderma has no cure. 

Difficulties that Scleroderma Patients Face 

Based on interviews conducted by Scleroderma United and NIH MedlinePlus Magazine, many patients share these common grievances with their scleroderma diagnosis: panicking when informed of their condition, feeling helpless due to a lack of support groups, and fear-based searching for information about the disease online. Many were not aware of the fact that a lot of information found online about scleroderma is inaccurate. Subsequently, patients’ families and friends do not actually understand their loved ones’ diagnosis. 

Another problem is financial burden, as the costs of scleroderma treatment and regular sessions with doctors are difficult to afford. This, coupled with the fact that health insurance only covers 10-50% of the cost of treatment, makes it virtually impossible for poor families to cope with a diagnosis. For patients who are not covered by health insurance, the treatment ranges from $1,000 to $67,000 per month, depending on the severity of organ involvement. Amputation is required for patients who have Raynaud’s disease and can cost anywhere from $20,000 to $60,000.

Due to the treatments needed to reduce the impact of scleroderma, the quality of life of these patients is considerably lower than that of the general population. In addition, this disorder places a considerable economic burden on healthcare systems and society as a whole. It is of paramount importance to inform the general public about scleroderma with an accurate portrayal of this condition.

References

Branch, NIAMS Science Communications and Outreach. “Raynaud’s Phenomenon.” National Institute of Arthritis and Musculoskeletal and Skin Diseases, 10 Apr. 2017, https://www.niams.nih.gov/health-topics/raynauds-phenomenon.

Fischer, Aryeh, et al. “Humanistic and Cost Burden of Systemic Sclerosis: A Review of the Literature.” Autoimmunity Reviews, vol. 16, no. 11, Nov. 2017, pp. 1147–54. DOI.org (Crossref), https://doi.org/10.1016/j.autrev.2017.09.010.

Hamaguchi, Yasuhito. “Drug-Induced Scleroderma-like Lesion.” Allergology International, vol. 71, no. 2, Apr. 2022, pp. 163–68. DOI.org (Crossref), https://doi.org/10.1016/j.alit.2021.08.005.

Henderson, Wendy. 7 Common Misconceptions About Scleroderma. 5 Oct. 2017, https://sclerodermanews.com/social-clips/common-misconceptions-scleroderma/.

“How Much Does Scleroderma Treatment Cost Cost?” CostHelper, https://health.costhelper.com/scleroderma-treatment-cost.html. 

Mayes, Maureen, and John Varga. “Scleroderma Overview and Causes.” National Scleroderma Foundation. 

“Scleroderma - Symptoms and Causes.” Mayo Clinic, https://www.mayoclinic.org/diseases-conditions/scleroderma/symptoms-causes/syc-20351952.