Scleredema in adults
Introduction
Introduction to adult scleredema Adultscleredema (adultscleredema) is a rare disease characterized by the sudden occurrence of diffuse symmetrical skin stiffness, which occurs after infectious diseases and can be relieved after several years. basic knowledge The proportion of illness: the incidence rate is about 0.002%-0.003% Susceptible people: no specific people Mode of infection: non-infectious Complications: acute renal failure
Cause
Causes of adult scleredema
(1) Causes of the disease
The cause is unknown. According to the majority of patients who develop infectious diseases (including pharyngitis, impetigo, cellulitis, measles, mumps, etc.), more than half of them are infected with Streptococcus, and whether this disease is an allergic reaction. The performance, or an autoimmune process, or a toxic disorder caused by microbial toxins in the matrix is still controversial, and the exact cause remains to be studied.
(two) pathogenesis
After the onset of infectious diseases, the pathogenesis is still unclear.
Prevention
Adult scleredema prevention
There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.
Complication
Adult scleredema complications Complications acute renal failure
1. Circulatory failure: often accompanied by obvious microcirculatory disorders such as pale complexion, cold skin, pattern, capillaries, filling time, prolonged heart rate, fast and slow heart rate, low heart sound, blunt arrhythmia, arrhythmia, heart failure, myocardial damage and cardiogenic shock.
2. Acute renal failure: When the disease is aggravated, it is accompanied by urinary oliguria or even no urine and other acute renal dysfunction.
3. Pulmonary hemorrhage: is the extreme performance of severe cases (1) dyspnea and sudden increase in sputum after aggravation of symptoms; (2) rapid increase of wet rales in the lungs; (3) foamy blood from the nose or mouth or endotracheal intubation liquid
Symptom
Symptoms of adult scleredema Common symptoms Skin is substantially non-... Dysphagia, inflammation, hair loss, sclerosis, parotid gland enlargement, arrhythmia, liver enlargement
Most cases often begin to develop symmetrical symmetry in the posterior neck and shoulders within a few days to 6 weeks after acute febrile illness, and quickly spread to the face, neck, scalp, chest and upper arms. Some cases can also affect the abdominal wall, buttocks and chest, the skin is substantially non-depressed and hard, the normal skin lines disappear, skin color, brown or pale, the surface is waxy luster, and the normal skin is not bordered. Clear, local feelings as usual, no atrophy, inflammation, pigment changes and hair loss, etc., the degree of swelling is often the heaviest neck, shoulders, back and face, touched with a hard skin-like feeling, such as the lack of facial expression It has a mask-like appearance, involving the oropharynx and tongue, the tongue becomes larger and dysphagia; if the neck is involved, the neck is inconvenient; if the chest wall is involved, the inhalation is limited, and in a few cases there may be hepatomegaly, skeletal muscle. And myocardial involvement, arrhythmia, pleural, pericardial and peritoneal exudation, occasionally lesions such as parotid swelling.
The disease usually improves within 3 to 6 months after the onset of the disease, most of which disappears within 2 years, and a few cases continue to retreat, or only partially improve, and maintain the residual hard zone for a long time without complete recovery. There are diabetes, and some cases can recur.
Examine
Adult scleredema examination
Some patients had an increase in anti-"O", and other items were abnormally irregular.
Histopathology: the epidermis is normal, the dermis is thickened about 3 times compared with normal. The collagen bundle is thickened and separated by a transparent cavity. The deposition of non-sulfate-hydrochloric mucopolysaccharide in the cavity is confirmed, the perivascular blood is slightly infiltrated, and the skin appendage is more. Without shrinking, it can be seen by electron microscopy that the collagen fibers of the hyperplasia are uniform in thickness, arranged in a bundle, accompanied by the accumulation of substances in the excess microfibers and the small volume and inactive fibroblasts.
Diagnosis
Diagnosis and diagnosis of adult scleredema
According to the skin lesions, they are often applied to the back neck or shoulders, and rapidly develop to the face, chest, back, upper arms, etc., and the progressive symmetry diffuse skin becomes hard, without atrophy, inflammation, pigmentation and hair loss. The local feeling is normal and the diagnosis can be determined.
The disease mainly needs to be differentiated from scleroderma. The initial stage of scleroderma is more common in the face and distal extremities. The skin color is darkened or mixed with pigmentation spots, sweat glands, sebaceous gland dysfunction, mane detachment, Raynaud's phenomenon. Common, histopathology shows epidermal atrophy, collagen fiber irregular hyperplasia, swelling, homogenization, hyaline degeneration, eosin and Masson 3 color staining is extremely deep, inflammatory cells infiltration more, appendages are atrophy, progressive progressive It is difficult to ease.
