bubble_chart Concept Chinese medicine believes that this condition falls under the categories of "Bi syndrome," "skin impediment," "migratory impediment," etc. Suwen‧Bi Lun states: "When Bi is in the bones, it causes heaviness; in the blood vessels, it causes blood stagnation and lack of flow; in the tendons, it causes inability to stretch; in the muscles, it causes numbness; and in the skin, it causes blockage." Chao's Zhubing Yuanhou Lun states: "Bi syndrome... its symptoms include thick and stubborn muscles, or muscle soreness... it is caused by blood qi deficiency leading to the invasion of wind-dampness, which results in this disease. If it persists for a long time without healing, it enters the meridians, affects the yang meridians, and can also lead to the inability to move the limbs." From this, it can be seen that the disease cause and mechanism of scleroderma are due to insufficient qi and blood, weak defensive qi, external contraction of wind-dampness, blockage between the skin, muscles, tendons, bones, and blood vessels, leading to disharmony between nutrient and defensive qi, Jingmai blockage, poor circulation of qi and blood, and even complete blockage, zang-fu organ dysfunction, and over time, damage to yang, depletion of qi, and deficiency of both the spleen and kidneys.
bubble_chart Modern Research
Scleroderma is a connective tissue disease characterized by skin hardening. It is generally divided into two types: systemic sclerosis and localized scleroderma. The former is often referred to as progressive systemic sclerosis, which typically includes two main subtypes: acrosclerosis and diffuse sclerosis. The disease progresses slowly, with clinical manifestations mainly including skin hardening in the form of droplets, patches, or bands, commonly occurring on the face, neck, chest, abdomen, back, and buttocks. Band-like hardening often occurs on the scalp, while droplet-like hardening is more common on the trunk, usually distributed in clusters with clear edges. Systemic sclerosis involves extensive skin changes and often affects the entire body, potentially leading to internal organ damage. Diffuse sclerosis progresses rapidly, with skin lesions spreading throughout the body and more severe internal organ involvement, while acrosclerosis shows the most pronounced skin changes in the limbs.
The disease often begins with Raynaud's phenomenon, accompanied by low-grade fever, general lack of strength, and joint pain. Skin disease changes can be divided into late stage [third stage], starting with edema, followed by hardening, and finally atrophy. Generally, there is no internal organ damage, and the prognosis is good. However, for those with internal organ involvement, the 5-year survival rate is 36% to 37%, with a worse prognosis for male and elderly patients.
The cause and pathogenesis of the disease are not fully understood, but it is generally believed to involve the following factors:
- Immune mechanisms: Most patients with this disease have various immune abnormalities and often coexist with other connective tissue diseases or autoimmune diseases.
- Vascular pathology: Vascular changes in scleroderma include Raynaud's phenomenon, early edema stage, telangiectasia, and capillary abnormalities observed under ultramicroscopy and nail bed examination, as well as widespread vascular lesions affecting internal organs.
- Collagen metabolism disorder: The increase in collagen connective tissue in this disease is due to increased collagen synthesis by fibroblasts.
[Diagnosis]
Main symptoms
- Skin symptoms: In the initial stage [first stage], unexplained edema and symmetrical diffuse hardening occur on the back of the hands and upper eyelids; in the advanced stage, skin hardening and finger flexion contractures develop.
- Limb symptoms: Raynaud's phenomenon is common; ulcers and scar formation at the extremities; multiple arthralgia or arthritis.
- Difficulty swallowing, pulmonary fibrosis, and difficulty breathing.
Pathological manifestations
Skin biopsy from the extensor side of the forearm shows collagen fiber swelling or fibrosis, with similar changes observed in the vascular walls.
bubble_chart Pattern Analysis - Qi Stagnation and Blood Stasis︰Localized or diffuse hardening of the skin, or swelling of the eyelids, face, and pyogenic hand dorsum carbuncle, with local pallor, cyanosis, coldness, burning, itching, and Raynaud's phenomenon. Accompanied by emotional irritability, menstrual irregularities, or nausea and vomiting, gum bleeding, loose stools or alternating dry and loose stools. The tongue texture resembles lotus rhizome nodes, with thin white coating, and a wiry pulse. Treatment involves regulating qi and relieving depression, activating blood circulation, and resolving stasis. Modified Peony and Gardenia Peripatetic Powder is used, including 10 grams each of Moutan Bark, Gardenia, Bupleurum, Chinese Angelica, Peony Root, Carthamus, dried Earthworm, Sichuan Curcuma Root, and Dragon's Blood Resin. Salvia and Suberect Spatholobus Stem, 30 grams each, and Cicada Slough, 6 grams. For qi deficiency, add 30 grams each of Tangshen and Astragalus Root. For damp stagnation and loose stools, add 15 grams each of White Atractylodes Rhizome and Indian Bread Tuckahoe. If symptoms include low-grade fever, aversion to cold, body and muscle pain, cough with thin phlegm, absence of thirst, soft stools, diffuse or localized hardening of the skin with a waxy sheen, or even atrophy, limited joint mobility, difficulty opening the mouth, absence of sweating or profuse sweating, a pale red tongue with thin white coating, and a rapid pulse, treatment involves ventilating the lungs to dispel cold, harmonizing nutrient and defensive aspects, and unblocking collaterals to resolve stasis. Schizonepeta and Saposhnikovia Toxin-Vanquishing Powder can be used with additions such as Cinnamon Twig, Peony Root, Chinese Angelica, Carthamus, Black-Tail Snake, Earthworm, Flowering Quince Fruit, Salvia, and Coix Seed.
- Yang Deficiencyblood Stasis︰Eyelid, facial, and pyogenic hand dorsum carbuncle with tight swelling, inability to make a tight fist, localized hardness, skin often appearing pink or mottled black and white, accompanied by fear of cold, cold limbs, joint pain, soreness and weakness in the lower back and knees, decreased libido, loose teeth, hair loss, poor appetite, absence of thirst, loose stool, swollen or enlarged and moist tongue. Tongue texture pale, coating gray and sluggish, pulse deep, thin, and weak. Treatment principle: warming the kidney to dissipate cold, invigorating blood and resolving stasis. Modified Yang-Harmonizing Decoction: Prepared Rehmannia Root 30g, degelatined deer-horn 20g, stir-fried white mustard seed 10g, Cassia Bark 10g, honey-fried Ephedra 10g, Dried Ginger 10g, Chinese Angelica 10g, Carthamus 10g, Prepared Liquorice Root 10g, suberect spatholobus stem 30g, Pyrola Herb 30g. For severe fear of cold and cold limbs, add prepared Aconite Lateral Root 15g and Dodder Seed 20g; if accompanied by mental fatigue and lack of strength, with a pale complexion, add Astragalus Root and Tangshen 30g each; for loose stool and greasy coating, add raw Coix Seed 30g, Atractylodes Rhizome 10g, and Floweringquince Fruit 15g; for localized hardness, add vinegar-prepared turtle carapace, kelp, and seaweed 15g each; for significant joint pain, add Gentian, Chinese Taxillus Herb 15g each, and Black-Tail Snake 6g.
- Qi Deficiency and Blood Stasis︰Skin lesions vary in severity, with pale or dull discoloration, cold and numb limbs, accompanied by mental fatigue and lack of strength; dizziness, poor appetite, weight loss, muscle pain, palpitation, and shortness of breath. The tongue is pale with a purplish lotus rhizome node and thin white coating, and the pulse is thin and rough. Treatment involves tonifying and replenishing qi and blood, as well as unblocking the vessels and resolving stasis. Modified Middle-Tonifying Qi-Replenishing Decoction is used: Codonopsis Root, Astragalus Root, and Prepared Rehmannia Root, 30g each; Prepared Liquorice Root, 10g; White Atractylodes Rhizome and Chinese Angelica, 15g each; Dried Tangerine Peel, Sichuan Curcuma Root, Carthamus, Salvia, Cinnamon Twig, Cyperus, and Suberect Spatholobus Stem, 10g each; Bupleurum, 6g. For muscle soreness, add Achyranthes Root, 15g, Gentian, 30g, and Pubescent Angelica, 10g. If the condition primarily affects the upper body, add Lonicera, 30g, and Turmeric, 10g. If it mainly affects the lower body, add Achyranthes Root and Prepared Aconite Lateral Root, 10g each. For widespread lesions, add Honey-Fried Ephedra and Cervi Deer-Horn Glue, 10g each.