sagging eyelids
Introduction
Introduction Blepharochalasis syndrome, also known as dermatolysis palpebrarum, atrophic ptosis atrophica, is a rare eyelid disease characterized by recurrent episodes of eyelid edema in adolescents. Thin, elastic disappearance, increased wrinkles, color changes, and can be associated with clinical manifestations of lacrimal gland prolapse, ptosis and cleft palate. In 1807, Beer first described the disease. In 1896, Fuchs called it eyelid retardation. Because the syndrome affects the appearance of the eyelids, it is the main reason for patients to require treatment. The understanding of their clinical manifestations and pathogenesis can help to adopt appropriate methods for treatment.
Cause
Cause
Causes of eyelid relaxation:
The etiology and pathogenesis of this disease are still unclear. Although there is a background description of autosomal dominant inheritance in the literature, most patients are sporadic cases with no family history. At present, it is believed that various factors synergistically lead to the onset of the disease. This disease has both congenital septum, weak fascia ligament development, and a history of acquired inflammation. Long-term repeated chronic inflammation caused swelling and swelling of the lacrimal gland, leading to further relaxation of the circulatory disorder, and the condition of lacrimal gland prolapse is gradually intensified, and the skin is simultaneously relaxed and degenerated. Inflammation swelling can also be accompanied by rupture and fissure of the aponeurosis of the diaphragm, resulting in aponeurotic ptosis. Histopathological examination of the skin and subcutaneous tissue of patients with hypertrophic and atrophic type showed that HE staining showed high edema of dermis and subcutaneous tissue, subcutaneous focal lymphocyte infiltration, collagen fibrosis, and muscle fiber atrophy. Special staining showed that the elastic fibers were reduced and broken, and IgA deposition was observed around them, suggesting that the pathological process may be autoimmune response mediated by IgA autoantibodies. Immunohistochemistry and electron microscopy showed that the target antigen bound by the autoantibody was mainly elastic binding protein and fibronectin. The elastic binding protein is a key component for maintaining the normal elastic state of tissues such as skin, so it can be considered as a birth defect of the elastic binding protein gene or The destruction of this protein by acquired factors is an important factor in the pathogenesis of this disease.
Examine
an examination
Related inspection
Eyelid examination ophthalmology
Examination and diagnosis of eyelid relaxation:
The syndrome is mainly seen in young women, and men also have an onset, with a male to female ratio of about 1:5. Patients can develop shortly after birth, but most of them occur in the 10 to 18 years old, and do not develop after puberty. About 80% of patients before the age of 20. Most of the lesions are bilateral, but there are also reports of unilateral onset. The patient has been physically healthy and there is generally no positive finding in the physical examination. Early clinical manifestations of recurrent episodes of unexplained recurrent epithelial cutaneous angioedema, edema persisted for 2 to 3 days and resolved spontaneously, against histamine drugs and corticosteroids, the frequency and extent of seizures between different patients and the same patient Different periods are different. Lower eyelids are only involved in severe cases, and unilateral morbidity is rare. Most patients often develop eyelid relaxation after emotional fluctuations or damage to the body. As the course of the disease progresses, the frequency of edema is gradually reduced. Finally, most patients can enter a relatively stationary phase. At this time, repeated edema and long-term pathological processes have damaged the tissue structure of the eyelids. Many patients have typical clinical manifestations. There may be sagging and sagging of the upper eyelid, so that the visual field cannot be completely lifted up, and the eyelids are atrophied and thinned, accompanied by telangiectasia and pigmentation. Due to the retraction of the lateral ligament, the transverse diameter of the cleft palate is shortened and the external condyle is blunt deformed. Can accompany or leave lacrimal gland prolapse, fat sputum, sputum, valgus and so on.
The incidence of orbital relaxation is low, and its clinical classification has long been controversial. Sichl classifies eyelid relaxation into type 3, low-tension, fatty and paralytic. Some scholars in China attribute it to the ectopic lacrimal gland, and therefore are divided into lacrimal gland prolapse type and dislocation lacrimal gland type. Custer et al. advocated dividing it into type 2, hypertrophic and atrophic according to clinical manifestations. It is believed that hypertrophy is mainly caused by dysplasia of the sacral septum, and fat sputum is caused by repeated inflammatory stimulation. The above sputum is full of hypertrophy as the main feature, and surgical treatment is used to strengthen sputum. Separated by the main. Atrophic type is caused by long-term chronic inflammation, soft tissue atrophy, thin skin lesions, and the above-mentioned sag and wrinkles are the main features. Surgical treatment is mainly to remove excess skin, and it is not recommended to open the septum and remove fat. This classification has certain guiding significance for clinical treatment programs and has been accepted by most scholars. Atrophic cases have been reported in foreign literature. Domestically, the type of hypertrophy is mainly accompanied by lacrimal gland prolapse. Lacrimal gland prolapse is an important sign of orbital relaxation syndrome. In the past, the causal relationship between lacrimal gland prolapse and eyelid relaxation was unclear. From the perspective of clinical pathology, the occurrence of both has its common mechanism. Lacrimal gland prolapse mainly manifests as double upper eyelids, and there are also unilateral ones. The upper eyelid area is red and swollen, and the condition is aggravated and there is heavy discomfort when fatigue occurs. Skin atrophy and thinning, slack and weakness, sagging can cover the external corner. The palpation of the palate is like a tongue-like mass slipping back. In the lower part of the lacrimal gland, the eyelids can be turned over to see the prolapsed lacrimal gland mass under the supracondylar conjunctiva. The skin of the external palate is degenerated, pigmented, and sees a clear boundary. Occasionally accompanied by heavy lips and aponeurotic ptosis.
Diagnosis
Differential diagnosis
Symptoms of eyelid relaxation and confusion:
Eyelid relaxation and upper lip thickening: characterized by loose eyelids and progressive thickening of the upper lip, it is called eyelid relaxation-upper lip hypertrophy syndrome, and some patients have goiter, also known as eye-mouth-thyroid syndrome. From the early onset, the eyelid edema began, after repeated episodes of eyelid skin relaxation, wrinkling, accompanied by telangiectasia, severe eyelid ptosis. From the infants and young children, the lips are repeatedly swollen, and the lips become fibrotic due to inflammation and become thicker into lips. Simple thyroid gland and swelling occur during puberty.
Upper eyelid thickening and relaxation: The head of patients with thick skin periosteal disease is a retrograde cranium, especially the upper eyelids are thick and slack, the ears and lips are also thick, especially large, and the skin of the hands and feet is also hypertrophic. The bones of the extremities and the phalanx are hypertrophied, the fingers and toes are sick, and the sacs and knee joints are effusion. The patient has pain in his limbs and his movements are awkward.
Eyelid closure is incomplete: the upper and lower eyelids cannot be completely closed, causing partial eyeball exposure, also known as rabbit eye. According to the clinical manifestations of the eye, it can be clearly diagnosed.
Eyelid drooping: also known as "hanging down." Due to insufficiency or disappearance of the levator palpebral function, or some or all of the upper jaw can not be lifted, the upper jaw is in a drooping position. Divided into complete and partial, monocular or binocular, congenital and acquired, true and false. If congenital, check whether: 1 simple ptosis (lifting or disappearing of the diaphragm function); 2 ptosis with upper rectus muscle function weakened; 3 ptosis with other ankle deformities, such as internal hemorrhoids Skin, etc.; 4 ptosis combined with (Marcus-Gunn) mandibular blinking movement phenomenon. If the nature is acquired, check whether: 1 traumatic eyelid or craniocerebral injury, or cervical sympathetic nerve injury; 2 disease such as myasthenia gravis; 3 mechanical such as trachoma sacral infiltration, or loss of support force of eyelids, If there is no eyeball. When there is suspected myasthenia gravis, it can be used as a test for neostigmine. When it is suspected of sympathetic drooping, it can be used as a test for hydroxyamphetamine.
