Contents 1 Pinyin 2 English reference 3 Disease alias 4 Disease classification 5 Disease overview 6 Disease description 7 Symptoms and signs 8 Cause of disease 9 Pathophysiology 10 Diagnostic examination 11 Treatment plan 12 Related sources attached: 1 Treatment of scleredema acupoint 1 Pinyin
yìng zhǒng bìng 2 English reference
scleredema 3 disease alias
Adult scleredema, Buschke scleredema 4 disease classification
Department of Dermatology and Venereology
5 Disease Overview
Scleredema is also known as adult scleredema. The cause is unknown and may be related to infection, especially streptococcal infection. Reported complications of diabetes and multiple myeloma.
Key points for diagnosis: 1. It is more common in young adults and more common in women. 2. There is a history of infection several weeks before the onset of illness. 3. The skin lesions originate on the head, neck and back, are symmetrical, and have unclear boundaries. The skin of the affected area is smooth and pale, with low skin temperature. The dermis is swollen and hard like a wooden board. It is adherent to the subcutaneous tissue and cannot move. The surface is not concave. If the face and oropharynx are involved, a mask-like expression will appear, and it will be difficult to open the mouth and swallow. 4. The skin lesions gradually develop toward the shoulders, upper arms, and upper trunk. 5. The condition reaches its peak in 2-4 weeks and lasts for several months or years before resolving naturally. Leave no trace. 6. Histopathology showed significant thickening of the dermis, thickening of collagen, and fibrosis of subcutaneous fat. There are gaps between collagen bundles.
Key points of treatment: 1. Early anti-infective treatment. 2. There is no specific treatment for skin lesions. Physical therapy, local injection of hyaluronic acid, plasmin, and corticosteroid preparations can be tried. 6 Disease Description
Scleredema is also known as adult scleredema and Buschke’s scleredema. It was first reported by Piffard in 1876 and described in detail by Buschke in 1902. About half of the cases of this disease are younger than 20 years old, so the name of adult scleredema is not accurate enough. It is characterized by diffuse non-pitting swelling and hardness of the skin, which mostly resolves naturally. 7 Symptoms and Signs
This disease is rare. There is occasional family history. There are more females than males, 29% are those before 10 years old, 22% are those between 10 and 20 years old, and the rest are adults. Although it can occur naturally, most patients (65% to 90%) have a history of infection a few days to 6 weeks before onset, such as influenza, tonsillitis, pharyngitis, measles, mumps, scarlet fever, impetigo, or cellulitis. Some cases have experienced trauma. Occasionally there are prodromal symptoms such as mild fever, fatigue, muscle pain and joint pain after infection and before the onset of disease, and punctate erythema is occasionally seen on the skin before hard swelling occurs. The rash starts on the head, face, neck or back, is symmetrical on both sides, appears swollen and hard, and has unclear boundaries with normal skin. The surface of the skin lesions was smooth, pale, and cool, with normal hair and pigment, and no ulcers or atrophy. 8 Causes of the disease
The cause of this disease is unknown. It has been proposed that this disease is caused by inflammatory obstruction of lymphatic vessels, peripheral nerves or pituitary gland functional disease. Because this disease is often secondary to infection, especially after streptococcal infection, Curtis et al. believe that streptococcal infection can cause an increase in mucopolysaccharides, sensitize collagen fibers, and produce autoimmunity. Some people applied estrogen to the skin of nude mice, resulting in non-pitting edema, and suggested that the disease is related to estrogen. Some stubborn cases are complicated by moderate to severe diabetes, but the skin lesions are ineffective in treatment with anti-diabetic drugs such as insulin and phenformin. Some cases have a history of trauma and multiple myeloma. 9 Pathophysiology
1. Pathogenesis
Because this disease is often secondary to infection, especially after streptococcal infection, Curtis et al. believe that streptococcal infection can cause an increase in mucopolysaccharides. , sensitizes collagen fibers, produces autoimmunity and causes disease. Some people applied estrogen to the skin of nude mice, resulting in non-pitting edema, and suggested that the disease is related to estrogen. Some stubborn cases are complicated by moderate to severe diabetes, but the skin lesions are ineffective in treatment with anti-diabetic drugs such as insulin and phenformin. Some cases have a history of trauma and multiple myeloma.
2. Pathological changes
The epidermis is basically normal.
The dermis is thickened, which can be 3 times thicker than normal, and the collagen is thickened and separated by clear gaps, creating the so-called "collagen window".
The epidermis is basically normal. The dermis is thickened, which can be 3 times thicker than normal, and the collagen is thickened and separated by clear gaps, creating the so-called "collagen window". Subcutaneous fat is replaced by dense collagen bundles. The sweat gland secretion ducts moved up to the upper or middle dermis, and the number of fibroblasts did not increase. Mast cells increased and there was mild cell infiltration around the blood vessels. In most early cases, hyaluronic acid is found between collagen bundles, especially in the "window area", using histochemical staining (such as colloidal iron staining). Metachromatic staining with phenol violet and toluidine blue (most obvious at pH 7.0, weaker at pH 5, and lacking at pH 1.5) suggests the presence of only non-sulfate acidic mucopolysaccharides. 10 Diagnostic tests
Laboratory tests: ESR was moderately increased, and serum protein was mildly abnormal, but not specific. The anti-"O" value is increased, especially in children. Also see IgG and IgA paraglobulinemia.
Electron microscopy has revealed numerous interfibrillar substances and collagen fiber coagulation. When the tongue, myocardium and skeletal muscles are involved, edema of the muscle fascicles and striations of the striated muscles disappear.
The autopsy of 1 case revealed diffuse edema in the heart, liver, and spleen, as well as pleural cavity and pericardial effusion.
According to the clinical characteristics and histopathology, the diagnosis of this disease is not difficult, but it should be distinguished from the following diseases: ① Localized or systemic scleroderma. Localized cases have a slow onset and the affected area is ivory in color. , the boundary is clear and the edge is lavender; Raynaud's phenomenon is often present in systemic scleroderma. 11 Treatment plan
In addition to radical cure of existing infections or lesions, other treatments are supportive or symptomatic. The reported therapies include physical therapy (such as massage), physical therapy (such as hot bath, ultraviolet light, diathermy, etc.), injection of hyaluronic acid, plasmin, and corticosteroids into local skin lesions, oral corticosteroids, estrogen, etc. Diols, pituitary hormones, thyroid preparations, calcium sodium edetate and para-aminobenzoic acid, but all have no definite effect. Radiation therapy may also be tried. 12 Related sources
Dermatology Acupoints for treating scleredema Hand Shaoyang Sanjiao Meridian
Diseases and other diseases in the parts it passes through. Such as: hard and full lower abdomen, bloating, difficulty urinating or enuresis, edema, deafness, tinnitus, sore throat, red and painful eyes, cheek... Feng Shui
) is the name of the disease. It is one of the edema diseases, also known as wind syndrome. "Zhongzang Jing·On the Life and Death of Edema Pulse Syndrome" and "Treatise on the Causes and Symptoms of Diseases·Esema Diseases" list Feng Shui as... Triple Burner Recruitment
Liver, kidney, spleen and stomach diseases, etc.: such as abdominal distension, firmness and pain , lower abdominal cramps, galloping, edema, urinary retention, enuresis, spermatorrhea, impotence, irregular menstruation, metrorrhagia, vaginal discharge... Duanda
Liver, kidney, spleen and stomach diseases, etc.: such as abdominal distension and firmness Pain, abdominal cramps, galloping, edema, urinary retention, enuresis, spermatorrhea, impotence, irregular menstruation, metrorrhagia, vaginal discharge... Fengchi