Pachydermoperiostosis
Introduction
Introduction to thick skin periostosis Pachyermoperiostosis, also known as cutaneous hypertrophic periosteal hyperosteogeny and Touraine-Solente-Gole syndrome, may be autosomal dominant, and secondary hyperproliferative osteoarthrosis. Often secondary to a variety of chronic and malignant neoplastic diseases, the pathogenesis is still unclear. Primary periosteal hyperplasia is mainly seen in men, which may be an autosomal dominant genetic disease with different penetrance. Such cases have been reported in the country. Secondary periosteal hyperplasia may also be a genetic disorder, but often caused by severe liver disease, bronchial lung cancer or epithelioid adenocarcinoma, bronchiectasis, lung abscess or by gastric cancer, esophageal cancer, thymic cancer. basic knowledge Sickness ratio: 0.001%-0.002% Susceptible people: no specific population Mode of infection: non-infectious Complications: pleurisy cardiovascular disease
Cause
Causes of thick skin periosteal disease
The primary may be autosomal dominant, secondary, also known as hyperproliferative osteoarthrosis, often secondary to a variety of chronic and malignant neoplastic diseases.
Prevention
Prevention of thick skin periosteal disease
Mainly for the prevention of various factors that may lead to thick-skinned pericarposis. The primary primary disease of this disease may be autosomal dominant inheritance. Therefore, early detection, early diagnosis and early treatment are important for indirect prevention of this disease. This disease is often secondary to various chronic and malignant neoplastic diseases. Therefore, active treatment of the primary disease is necessary for prevention.
Complication
Thick skin periosteal complications Complications pleurisy cardiovascular disease
Primary hypertrophic osteoarthrosis can be complicated by joint effusion, causing joint pain, swelling and inability to walk. Myelofibrosis can affect hematopoietic function, leading to complications such as anemia, leukopenia, and thrombocytopenia. Gastrointestinal proliferative lesions and chromosomal abnormalities, secondary hypertrophic osteoarthritis can be complicated by lung and pleural diseases, cardiovascular diseases and extrathoracic diseases.
Symptom
Symptoms of thick-skinned pericarposis Common symptoms Skin hypertrophy Upper eyelid thickening Relaxation Horizontal stripes Deepening Joint effusion Diffuse Periosteal thickening refers to hypertrophic cranial periostitis
1. Primary: more common in men, often in the short period after puberty, face, forehead, head skin hypertrophy, wrinkled, forehead changes are particularly prominent, the amount of horizontal stripes deepens, the head is a retrograde cranium, Eyelids, especially the upper eyelids, are thick and slack, the ears and lips are also thick, especially large, the skin of the hands and feet is also hypertrophy, the bones of the limbs and the phalanx are hypertrophy, the fingers and toes are sick, the sacs, the knee joint effusion, the patient's limbs are painful. The action is clumsy.
Primary people often occur shortly after puberty. The skin on the face, forehead, and scalp is thickened and wrinkled. Wrinkles and grooves on the forehead and cheeks, as well as thickened eyelids, give the patient an anxious and disappointed face. The wrinkles of the scalp form a scalp. The skin of the hands and feet is also thickened but not wrinkled. The sebaceous glands on the face and scalp are active, the secretion is significantly increased, and hyperhidrosis occurs in the hands and feet, causing the patient to feel uncomfortable.
The fingers (toes) and long bones of the limbs are thickened to make the hands and feet behave like a shovel. The forearms and calves are cylindrical due to thickening of the periosteum, and the ankles and knees are swollen with fluid.
The lesions of the skin and joints gradually increase, and after 5 to 10 years, they remain unchanged for the rest of their lives, and occasionally progressively worse. Excessive hyperplasia of sebaceous glands. In some cases, the hair on the head and genitals may be sparse. Men may develop breast development. Whether there is endocrine barrier is inconclusive. Many patients develop mental retardation, and in severe cases, they can cause labor loss and shorten their life.
2. Secondary: After the middle age, the disease is more common in women, the skin changes are not significant, the bone lesions are obvious and fast, and the pain is conscious. After the primary disease is alleviated, the bone and skin lesions are alleviated. According to the typical changes of skin and bone can be diagnosed, X-ray examination of the tibia, tibia, tibia, ulna and other proliferative periostitis, diffuse periosteal thickening.
Secondary people are mainly seen in men between the ages of 30 and 70. The lesions in the bones are significant, and they develop rapidly and often have pain. However, the lesions of the skin are not significant, and individual manifestations can also be significant. If the primary lesion is effectively treated, it can reduce the bone and skin lesions.
Examine
Examination of thick skin periosteal disease
X-ray examination showed proliferative periostitis such as humerus, humerus, humerus and ulna, and diffuse periosteal thickening.
Diagnosis
Diagnosis and differentiation of thick skin periosteal disease
Diagnostic points:
First, the primary: 1. After puberty. 2. The forehead and cheek skin are obviously thickened, wrinkles appear, the scalp is thickened to form the scalp, the skin of the hands and feet is thickened, no wrinkles, often sweaty; often accompanied by obvious clubbing. 3. The extremities are thickened in the shape of a periosteum, and the sacral and knee joints are swollen. 4. Histopathology shows that long bone is predominantly proliferative periostitis with diffuse irregular periosteal thickening. 5. Can be accompanied by mental retardation. 6. The disease can be stable and not continue to develop 5 to 10 years after the onset of illness.
Second, secondary: 1. The incidence of middle-aged and elderly. 2. The periosteal thickening is fast and obvious, often painful, but the skin lesions are lighter. 3. Histopathology is the same as primary.
The disease should be differentiated from acromegaly and thyroid acromegaly.
