What is skin fibrosis

Fibrosis of the skin

Fibrosis of the skin or scleroderma , a Greek word derived from the word "sclero" meaning hard, and "derma" meaning skin, ^{[1] and it} is a chronic immune disease in which immune cells attack various body tissues, which leads to the death of these cells And its destruction, ^[2] where the body produces excess amounts of collagen than it needs, which leads to hardening of the surface of the skin, and a decrease in its elasticity. ^[3]

The severity of the disease varies from very mild to life threatening. The number of people with skin fibrosis in the United States of America is 300,000, and since the symptoms of this disease are similar to the symptoms of other immune diseases, its diagnosis is difficult, so it is possible that there are a number of cases that have been misdiagnosed, and cases that have not been diagnosed. It is worth noting that skin fibrosis affects women more than men with a ratio of (1: 4), respectively, [1] and usually appears after the age of thirty years of age, [4] and it is a non-contagious disease and not a type of cancer, [1] and it is possible that Affects the internal body systems; Like the heart, lungs, kidneys, and gastrointestinal tract. [5]

Types of skin fibrosis

Dermatofibrosis is divided into two main types:

Systemic dermal fibrosis

Systemic Scleroderma affects both internal organs and the skin, and it is of two types: ^[2]

• Limited Cutaneous Fibrosis: Thickening and hardening of the skin is noted in the hands , forearms, legs, and feet.
• Diffuse Cutaneous Fibrosis: This affects larger areas of the body, such as the hands, whole arms, thighs, and torso.

In both types, the face is vulnerable. ^[2]

Limited skin fibrosis

Localized Scleroderma affects only the skin , and there are two types:

• Morphea: This type is characterized by the presence of thick spots ranging in diameter from an inch to six inches or more, and they are either one spot or several spots spread all over the body. These spots are light or dark compared to normal skin color, and are often accompanied by a loss of a layer of sebum beneath. ^[2]
• Linear Scleroderma: This type is characterized by the presence of thick lines of skin on the arm and feet on one side of the body only, and they are accompanied by a loss of the fat layer underneath, so the size of the affected side is smaller compared to the healthy side. ^[2]

The cause of skin fibrosis

The exact cause of skin fibrosis is still unknown, but the processes that permeate this disease and lead to its complications are clear. In the case of limited fibrosis, the body produces quantities of collagen in excess of its need in the affected areas, which leads to a change in the shape of the skin, and this also occurs in the case of systemic fibrosis that is accompanied by a defect in the immune system, and this is also accompanied by narrowing of the capillaries, which leads to the occurrence of something known as the phenomenon of Renault ^[2] It is a temporary narrowing of the blood vessels is aggravated in the cases of cold or stress due to lack of access sufficient amount of blood to different organs, leading to the patient numb feeling and coldness in the limbs, and turns to a sometimes blue . ^[6] In cases of skin fibrosis, the body also witnesses various inflammations. ^[7]

Factors that may affect the occurrence of skin fibrosis include the following:

• Age. ^[1]
• Sex. ^[1]
• Environmental factors: Environmental factors play a role in stimulating skin fibrosis, for example dealing with silica in mines, some chemicals and some drugs. ^[2]
• Genetic factor: Researchers indicate that a certain gene may be present in the body that increases the likelihood of developing skin fibrosis. ^[1]
• Ethnicity: Native United States or (Native Americans) and Africans are more likely to have skin fibrosis than others. ^[2]

The effect of dermal fibrosis on the body's organs

• Blood vessels: their impact appears in the form of inflammation, dilation and contractions in them, ^[8] which is what we see in the phenomenon of Raynaud. ^[9]
• Mouth: Patients may experience a reduction in the size of its opening, which is called microstomia or small mouth, which makes movement of the mouth and lips difficult, in addition to the difficulty of caring for oral health, ^[10] as well as dry mouth, jaw pain, and gum disease . Some patients may develop what is known as Sjogren's syndrome, which affects the salivary glands. ^[8]
• The hands : The fingers, joints and wrists are affected by many injuries, such as the Raynaud's phenomenon, which leads to infections and ulcers, ^[8] and calcinosis due to calcium accumulation in soft tissues, and hardening of the skin in the extremities. ^[11]
• Musculoskeletal system: the effect is in the form of pain and inflammation in the muscles, joints and ligaments. ^[8]
• Heart and lungs: The lungs may experience scarring, which is called pulmonary fibrosis , and the patient may develop pulmonary arterial hypertension, which is high blood pressure in the pulmonary blood vessels. ^[8]
• Kidneys: The crisis nephrotic of the most serious complications affecting the kidneys in the case of cirrhosis of the skin systemic, characterized by blood pressure rise suddenly. ^[8]
• The brain: the injury in this case is psychological, as patients suffer from anxiety and depression. ^[8]
• The digestive system: It is considered one of the organs most affected by cutaneous fibrosis after the skin, as the percentage of patients who have symptoms related to the digestive system is estimated between 75% - 90%. ^[8] Patients suffer from esophageal problems, difficulty swallowing, diarrhea, and constipation . ^[12]
• Eyes: if the patient develops Sjogren's syndrome; He will suffer from dry eyes , and this may lead to irritations and serious infections. It should be noted here that Sjogren Syndrome is an immune disease characterized by a deficiency in the functioning of the lacrimal and salivary glands, and affects approximately 20% of people with skin fibrosis. ^[13]
• Skin: It is the most important factor affected by skin fibrosis, as the patient suffers from increased thickness and dryness, a feeling of itching, sores, hair loss in the affected areas, an increase or decrease in pigmentation, and a decrease in the effectiveness of the skin in the secretion of sweat abroad. ^[14]
  

Treating skin fibrosis

There is no cure for skin fibrosis yet, but there are methods used to reduce the symptoms of the disease and the speed of its progression, ^[1] including the following:

• Medicines to relieve heartburn, such as Proton Pump Inhibitors, and drugs to improve bowel movements. ^[1]
• Non-steroidal anti-inflammatory drugs (NSAIDs) and corticosteroids such as prednisone. ^[15]
• Vasodilators, such as: Nifedipine, which reduce the chances of recurring Raynaud's phenomenon and ulcers in the extremities. ^[16]
• Use of colchicine. ^[17]
• Photochemotherapy (PUVA), and this treatment consists of a drug taken by mouth in addition to exposure to ultraviolet light. ^{[17] [18]}

References

1. ^ ^{A b t w c h x d} "What ' Is Scleroderma?" , Scleroderma Foundation , Retrieved 2017-6-21. Edited.
2. ^ ^{A b t w c h x d} "SCLERODERMA: And Overview The Causes" , Scleroderma Foundation , 2013-7, Retrieved 2017-6-21. Edited.
3. ↑ SUBCOMMITTEE FOR SCLERODERMA CRITERIA OF THE AMERICAN RHEUMATISM ASSOCIATION DIAGNOSTIC AND THERAPEUTIC CRITERIA COMMITTEE (May 1980), (PRELIMINARY CRITERIA FOR THE CLASSIFICATION OF SYSTEMIC SCLEROSIS, 58 .
4. ^ Philip J. Clements and Daniel E. Furst, Systemic Sclerosis 2nd Edition , Page 7. Edited.
5. ^ Philip J. Clements, Daniel E. Furst, Systemic Sclerosis 2nd Edition , Page 16.
6. ^ “Raynaud's Disease” , The Vascular Disease Foundation , 2012. Edited.
7. ^ Philip J. Clements, Daniel E. Furst, Systemic Sclerosis 2nd Edition , Page 8. Edited.
8. ^ ^{A b t w c h x d} "Scleroderma ( . SYSTEMIC Sclerosis): How To It Affects The Body" , Scleroderma Foundation , 2013, Retrieved 2017-6-21. Edited.
9. ↑ “Scleroderma (Systemic Sclerosis): How It Affects The Body-vascular System" , Scleroderma foundation , 2013, Retrieved 2017-6-21.
10. ↑ "Scleroderma (Systemic Sclerosis): How It Affects The Body-oral" , Scleroderma foundation , 2013, Retrieved 2017-6-21. Edited.
11. ↑ “Scleroderma (Systemic Sclerosis): How It Affects The Body-hands" , Scleroderma foundation , 2013, Retrieved 2017-6-21.
12. ↑ “Scleroderma (Systemic Sclerosis): How It Affects The Body- digestive systym and gastrointestinal tract” , Scleroderma foundation , 2013, Retrieved 2017-6-21. Edited.
13. ↑ "Scleroderma (Systemic Sclerosis): How It Affects The Body-eyes" , Scleroderma foundation , 2013, Retrieved 2017-6-21.
14. ↑ “Scleroderma (Systemic Sclerosis): How It Affects The Body-skin” , Scleroderma foundation , 2013, Retrieved 2017-6-21.
15. ^ Philip J. Clements, Daniel E. Furst, Systemic Sclerosis 2nd Edition , Page 25. Edited.
16. ^ Philip J. Clements and Daniel E. Furst, Systemic Sclerosis 2nd Edition , Page 27.
17. ^ ^{A b} Jale Y¨uksek1, Engin Sezer1, Dog˘an K¨oseog˘lu2 , and others. (2010), -Photodermatology, Photoimmunology & Photomedicine- Scleredema treated with broad-band ultraviolet A phototherapy plus colchicine , Page 257, Part 26.
18. ↑ Gary W. Cole (2016-8-8), “PUVA Therapy (Photochemotherapy)" , MedicineNet , Retrieved 2017-7-6.