Angiokeratoma
Introduction
Introduction to vascular keratomas Vascular keratomas, also called vascular keratomas, are skin diseases characterized by telangiectasia and hyperkeratosis of the dermis. Vascular keratomas occur in children or adolescents, and women are more common. There is often a history of frostbite or frostbite before the onset of illness. It has been reported that countless people in the same family are sick. Vascular keratomas This disease is divided into five types, namely, apex type, scrotum type, papule type, boundary type, and general type. The last type is a lipidoid disease. There are two kinds of primary damage: 1 is needle to corn rash or pimples, the surface is rough, keratinized, purple or dark purple, sometimes it can fade; 2 is a nodule, diameter is 2-8mm, surface keratin proliferation or It is sputum-like, purplish red or gray, with common dilated capillaries or blood stasis in the center, and is prone to bleeding after trauma. No symptoms, or can gradually heal. Some patients have acral cleft or frostbite. Serum immunoglobulin can be increased. The skin lesions of the generalized hair are symmetrically distributed on the limbs and trunk, and may be accompanied by pain. basic knowledge The proportion of illness: 0.001% Susceptible people: good for children or teenagers, more common for women Mode of infection: non-infectious Complications: cardiomyopathy
Cause
Cause of vascular keratoma
Lack of -galactosidase, the disease is associated with recessive inheritance.
Prevention
Vascular keratinoma prevention
Mainly prevention-based, timely detection of timely treatment.
Complication
Vascular keratinoma complications Complications cardiomyopathy
Diffuse somatic keratomas, which cause damage to the human heart, kidneys, and autonomic nervous system, usually die at 50 years of age due to cardiomyopathy and renal insufficiency.
Symptom
Symptoms of vascular keratinoma Common symptoms Pimples, blemishes, limbs, cold hair, rare nodules, pores, hyperkeratosis, paralysis
1.Mibelli vascular keratomas: skin lesions are 1 to 5 mm dark red or purple red round papules, the surface is scorpion-like, common on the back of the finger (toe) and elbow and knee, the patient's hands and feet often have cold hair.
2.Fordyce vascular keratomas: lesions are multiple small vascular papules, 2 ~ 4mm size, early skin lesions are red soft papules, late pale blue purple keratinized papules, lesions occur in the scrotum (Figure 1), sometimes occurs in female yin, more common in middle-aged and elderly.
3. Isolated vascular keratomas: early skin lesions are bright red soft papules, later turned blue-black, solid keratotic excessive papules, size 2 ~ 10mm, skin lesions are single, even several people, good For young people, most of them are found in the lower limbs.
4. Localized vascular keratomas: Individual lesions are single or occasional clumps of blood red papules and cystic nodules filled with blood, gradually becoming sickle and fused into single or several plaques If there are several plaques, they tend to be arranged in a line. The plaques increase with the patient's growth, and new lesions can occur. The size is often only a few centimeters. Occasionally there are quite large, localized keratosis. Tumors can be associated with port wine stains, cavernous hemangioma, and Klipper-Trenanay syndrome. This disease occurs mostly at birth, but a few also occur in children.
5. Diffuse somatic keratomas: more common in children and adolescents, skin lesions are a wide range of punctate telangiectasia papules, size 1 ~ 2mm, suspicion of purpura, some papules keratosis, but not as good Other vascular keratomas are obvious, lesions occur in the lower torso, lower extremities, scrotum, penis, armpits, ears and lips, hair thinning, endothelial cells, fibroblasts, dermal pericytes, heart, kidney and autonomic nervous system Deposition of glycolipids, usually at the age of 50, can cause death due to cardiomyopathy and renal insufficiency, common ankle edema, paralysis, burning sensation in the hands and feet and hypohidrosis, visible conjunctival and fundus vascular abnormalities, corneal development in 90% of patients Significant vortex-like opacity, 50% of patients with characteristic wheel-like cataract in the posterior capsule, white blood cells, serum, tears, skin fibroblasts, -galactosidase reduction, can confirm the diagnosis.
Examine
Examination of vascular keratomas
1.Mibelli vascular keratomas: early vasodilatation of the upper part of the dermis, part of the prolonged epidermal ganglion, old lesions, hyperkeratosis of the epidermis, some dilated capillaries completely surrounded by the epidermis, some blood vessels Expansion is not limited to the upper part of the dermis, but also in the middle of the dermis.
2. Fordyce vascular keratomas: same as Mibelli vascular keratomas.
3. Isolated vascular keratomas: the same as Mibelli vascular keratomas, occasionally visible or mechanical thrombosis in the dilated blood vessels.
4. Localized vascular keratomas: see varying degrees of hyperkeratosis, papilloma and irregular acanthosis, which are seen below the very dilated capillaries, or surrounded by papillary epidermis. In most cases, all the cavities contain only red blood cells, but even thin wall cavities filled with lymph fluid are seen, and some thrombus is seen in the dilated capillaries.
5. Diffuse somatic keratomas: the dermal papillary vasodilation, forming a cavity lined with endothelial cells and filled with blood, surrounded by hyperkeratosis and hypertrophic epidermis, electron microscopic endothelial cells, week Characteristic electron dense bodies are found in cells and fibroblasts.
Diagnosis
Diagnosis and differentiation of vascular keratomas
According to the clinical classification and performance, combined with the characteristics of skin lesions and histopathology can be diagnosed, white blood cells, serum, tears, skin fibroblasts, -galactosidase reduction, can confirm the diagnosis.
Isolated vascular keratomas should be differentiated from seborrheic keratosis, melanoma, pigmented basal cell carcinoma, and other hemangiomas.
