Loose and wrinkled skin

Introduction Dermatolysis, also known as cutis laxa, or generalized elastolysis, is a skin sagging disease caused by congenital defects in skin elastic fibers and can invade the body. Connective tissue, involving the normal structure and function of cardiovascular, respiratory, urinary and other organs. Usually divided into two types: innate and acquired. Congenital type is autosomal recessive or partially dominant. Acquired people have no genetic background, no family history, and are often based on secondary inflammation following skin inflammation.

Cause

(1) Causes of the disease

Congenital type is autosomal recessive or partially dominant. Acquired people have no genetic background, no family history, and are often based on secondary inflammation following skin inflammation.

(two) pathogenesis

Congenital type, due to congenital copper ion metabolism abnormality affects the concentration of elastase inhibition, causing the elastic fiber congenital development defects and disease. Obtained type, often due to skin inflammation secondary to this disease.

an examination

1. Congenital type: Skin symptoms are found shortly after birth. The first skin edema, after the appearance of skin loose, sagging wrinkled, neck, face, eyelid wrinkles most prominent, showing the appearance of early and old. The ptosis of the upper jaw can obstruct the line of sight, the sag of the lower jaw is formed by the valgus, and the skin of the abdominal wall and the neck is relaxed and overhanging. Other organs are damaged by elastic fibers, tissue relaxation, often sacral sputum, gastrointestinal diverticulum, mucosal prolapse and cardiovascular dilatation or aneurysm. In addition, there may be corneal annular hemangioma, lingual crack, uvula, tooth loosening, pharyngeal mucosa swelling, vocal cord relaxation, hoarseness, hair thinning and genital dysplasia. Common skin affected areas are mainly face, eyelids, ears and neck. It appears in the wrinkles of the wrist, knee, ankle, and abdomen.

2. Acquired type: usually adulthood, some may have skin inflammation first, after elastic tissue release and relaxation, such as eczema, polymorphous erythema, urticaria, angioedema, skin relaxation symptoms may occur, but no congenital type The characteristics of developmental disorders.

According to the clinical manifestations, the characteristics of skin lesions and histopathological features can be diagnosed.

Differential diagnosis

Shrinkage on the surface of the skin lesions under cold stimulation: single or clustered painful papules or nodules appear in the clinic, and skin leiomyomatosis should be considered when the surface of the lesion is shrunk under cold stimulation. Leiomyoma cutis is a benign tumor composed of skin fascia, mesothelial or vascular smooth muscle. There are few skin smooth muscles, mainly found in the hairy muscles near the hair follicles, the muscle fibers around the deep subcutaneous blood vessels, the smooth muscle bundles in the scrotum and the labia.

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