1 Western medical aetiology and pathology

Scleroderma literally means hard skin. It is a symptom of a group of diseases that involves the abnormal growth of connective tissue, which supports the skin and internal organs. In some cases, scleroderma occurs as hard, tight skin; in others, the damage goes deeper, affecting blood vessels and organs, such as the lungs, heart, liver and kidneys.

The cause of scleroderma is generally unknown and it may be the result of several factors working together. However, most researchers think the causes include autoimmunity, environmental exposure, genetics and infections.

T-cell activation plays a pivotal role in the immunopathogenesis. Lymphocyte processes are tightly controlled by molecules that activate either proliferation or apoptosis. Possibly an imbalance in apoptotic function or increasing autoreactive cells may lead to persistent autoreactive phenomena.¹

Patients with systemic sclerosis also have altered B-cell homeostasis characterized by increased numbers of native B cells and reduced memory B cells. Although memory B cells are decreased in number, they are chronically activated, possibly because of CD19 overexpression in B cells.² The immune system and inflammatory activity appears to mirror many other rheumatic disorders. In scleroderma, the disordered immune system stimulates fibroblasts to produce too much of the collagen found in lungs, skin and kidneys. In its milder forms, the effects of this build-up are limited to the skin and blood vessels. In its more serious forms, it also can interfere with the normal functioning of joints and internal organs.

Genetics may also be a predisposing reason why some people are at risk of scleroderma.

Exposure to some environmental factors may trigger the disease in people who are genetically predisposed to it. Suspected triggering factors include viral infections, certain adhesive and coating materials, and organic solvents such as vinyl chloride or trichloroethylene.

Women have a much higher risk of developing scleroderma than men. However, so far, the role of oestrogen or other female hormones has not been proven in the process of scleroderma.

When the lungs are involved, the alveoli and capillaries are damaged and destroyed, leading to pulmonary hypertension. The result is lower oxygen levels in the blood, which may lead to heart failure.

2 Diagnosis by Western medicine

Scleroderma has two main classifications: localized scleroderma that affects only certain parts of the body, and systemic sclerosis that affects the entire body. Both groups include subgroups, so the skin may show tightness, thickening and/or hardening on physical examination.

Diagnosis is made by laboratory tests and radiography:

3 Chinese medical aetiology and pathology

Scleroderma is a kind of autoimmune disease caused by sclerosis of the skin. There are two types or scleroderma: localized and systemic. Generally the process has three stages: red skin with swelling, sclerosis and atrophy. Chinese medicine calls this Pi Bi , or skin hardening.

Wind-Heat or Wind-Cold can invade the body, leading to pathogenic Heat or Cold in the Lung, or Warm diseases develop and impair Yin. This can then lead to deficient Fire singeing the Blood, causing Blood stasis which blocks the channels. As a result, Qi and Blood cannot flow smoothly through the channels, so damaging the skin. These pathogenic factors can also follow the channels into certain organs and impair their function.

Wind-Cold-Dampness can also invade the body. At the start, the pathogens stay in the Lung and cause skin thickness and swelling. The Cold-Dampness directly impairs the Spleen and can cause a Spleen function disorder. This leads to Qi deficiency and loss of the Qi’s function of warming the body and promoting Blood circulation in the blood vessels. If Qi deficiency prevents the Blood from flowing smoothly in the vessels, it can cause the Blood to leave the vessels and become Blood stasis. When the disease lasts for a long time, the pathogens continue to block the channels, affecting the smooth movement of Liver Qi and leading to Liver Qi stagnation. If Qi stagnates for a long time, this will transform to Fire and impair Yin. Yin deficiency and Qi stagnation can aggravate Blood stasis. Eventually, the process of the illness becomes Heat, Yin deficiency, Blood stasis and Qi deficiency.

4 General treatment principle

Pathogenic factors of the illness include Heat, Cold, Yin deficiency, Qi deficiency and Blood stasis. Heat, Cold, and Yin and Qi deficiency all cause Blood stasis or aggravate Blood stasis. Conversely, Blood stasis can aggravate the illness. No matter what the pathogenic factors are, the key pathological change is Blood stasis. Therefore, no treatment will succeed without removing Blood stasis.

5 Appendix

(2) Pulmonary hypertension

1 Liver Qi Stagnation with blood stasis

Clinical manifestations

Shortness of breath and difficulty breathing, distending pain in the chest, anxiety, depression and frequent sighing. The tongue is light red. The pulse is taut.

Treatment principle

Promote flow of Liver Qi, promote Blood circulation and remove Blood stasis.

Herbal treatment

GUA LOU XIE BAI BAI JIU TANG and XUE FU ZHU YU TANG variation

Trichosanthes Fruit, Chinese Chive and Wine Decoction, and Drive Out Stasis in the Mansion of Blood Decoction

Gua Lou Trichosanthis Fructus 12 g

Xie Bai Allii macrostemi Bulbus 12 g

Dang Gui Angelicae sinensis Radix 9 g

Sheng Di Huang Rehmanniae Radix 12 g

Tao Ren Persicae Semen 12 g

Hong Hua Carthami Flos 12 g

Chi Shao Paeoniae Radix rubra 12 g

Gan Cao Glycyrrhizae Radix 3 g

The herbs above are decocted in water for oral administration.

Acupuncture

Du-20 Baihui, BL-18 Ganshu, SP-10 Xuehai and LIV-3 Taichong.

Supplementary treatment

If the patient has depression, add Chai Hu Bupleuri Radix and Yu Jin Curcumae Radix, and needle Du-20 Baihui, Yintang (extra) and HE-7 Shenmen; if the patient has indigestion, add Ji Nei Jin Gigeriae galli Endothelium corneum and Shan Zha Crataegi Fructus, and needle Ren-12 Zhongwan.

2 Spleen Qi Deficiency with Blood Stasis

Clinical manifestations

Shortness of breath and difficulty breathing, pale facial colouring, fatigue and loose stool. There may be swelling in both ankles and palpitations. The tongue is pale with teeth marks. The pulse is deep and weak.

Treatment principle

Nourish Spleen Qi and remove Blood stasis.

Herbal treatment

SHEN LING BAI ZHU SAN and TAO HONG SI WU TANG variation

Ginseng, Poria and Atractylodis Macrocephalae Powder, and Four-Substance Decoction with Safflower and Peach Pit

Dang Shen Codonopsis Radix 12 g

Huang Qi Astragali Radix 12 g

Bai Zhu Atractylodis macrocephalae Rhizoma 9 g

Fu Ling Poria 12 g

Hong Hua Carthami Flos 12 g

Tao Ren Persicae Semen 9 g

Sheng Di Huang Rehmanniae Radix 9 g

Chi Shao Paeoniae Radix rubra 12 g

Chuan Xiong Chuanxiong Rhizoma 9 g

Dan Shen Salviae miltiorrhizae Radix 12 g

The herbs above are decocted in water for oral administration.

Acupuncture

BL-17 Geshu, BL-23 Pishu, ST-36 Zusanli and SP-9 Yinlingquan.

Supplementary treatment

If a patient has diarrhoea, but no stomach pain or no burning sensation with bowel movement, and the tongue is pale and the pulse is deep and slow, add Bu Gu Zhi Psoraleae Fructus and Wu Wei Zi Schisandrae Fructus, and needle SP-4 Gongsun and ST-25 Tianshu.

3 Liver and kidney yin deficiency with blood stasis

Clinical manifestations

Shortness of breath with difficulty breathing, chest pain, thirst and dry mouth, which may be accompanied by dry skin. The tongue is red with less coating or a fissure. The pulse is thready and rapid.

Treatment principle

Nourish Liver and Kidney Yin and remove Blood stasis.

Herbal treatment

LIU WEI DI HUANG WAN and DAN SHEN YIN variation

Six-Ingredient Pill with Rehmannia and Salvia Decoction

Sheng Di Huang Rehmanniae Radix 15 g

Shan Zhu Yu Corni Fructus 9 g

Mai Men Dong Ophiopogonis Radix 9 g

Wu Wei Zi Schisandrae Fructus 6 g

Mu Dan Pi Moutan Cortex 6 g

Fu Ling Poria 9 g

Dan Shen Salviae miltiorrhizae Radix 12 g

San Leng Sparganii Rhizoma 9 g

E Zhu Curcumae Rhizoma 9 g

The herbs above are decocted in water for oral administration.

Only gold members can continue reading. Log In or Register to continue

Share this:

• Click to share on Twitter (Opens in new window)
• Click to share on Facebook (Opens in new window)

Related

Related posts:

Basic immunology and immune system disorders Hashimoto’s thyroiditis Raynaud’s phenomenon Graves’ disease Crohn’s disease Cardiopulmonary disease and lupus

Stay updated, free articles. Join our Telegram channel

Join