Hereditary sclerosing heterochromia

Introduction to hereditary sclerosing cutaneous heterochromia Hereditary sclerosing cutaneous heterochromia (hereditarysclerosing poikiloderma) is an autosomal dominant connective tissue disease, and the pathogenesis is still inaccurate. Occurs in the wrinkles of the armpits, elbows, etc., and the lesions are characterized by systemic cutaneous heterochromia. The diagnosis relies on histopathology and there is currently no satisfactory therapy. It may also have a certain relationship with the life behaviors such as mental state and diet structure during pregnancy. Its histopathological features, homogenization of collagen fibers and reduction of elastic fibers in dermal tissue. basic knowledge The proportion of illness: 0.0015% Susceptible people: no special people Mode of infection: non-infectious Complications: vitiligo

Causes of hereditary sclerosing cutaneous heterochromia

Cause

Heraditary sclerosing poikeroderma is an autosomal dominant connective tissue disease. Skin lesions manifested as systemic cutaneous heterochromia, with hyperkeratosis and hardening of the skin at the site of the hair, and other visible keratosis of the palmar palpebra, clubbing and skin-limited calcium deposition.

Hereditary sclerosing cutaneous heterochromia prevention

A reasonable diet can take more high-fiber and fresh vegetables and fruits, balanced nutrition, including essential nutrients such as protein, sugar, fat, vitamins, trace elements and dietary fiber, with a combination of vegetarian and vegetarian foods. The complementary role of nutrients in food is also helpful in preventing this disease.

Hereditary sclerosing cutaneous heterochromia complications Complications

Generally no complications.

Hereditary sclerosing cutaneous heterochromia symptoms Common symptoms Skin dull skin heterochromia Skin hardening palm and toe skin... Epidermal keratinized finger (toe)

The disease occurs in the wrinkles of the armpits, elbows and other places. Skin lesions manifested as systemic cutaneous heterochromia, with hyperkeratosis and hardening of the skin at the site of the hair, and other visible keratosis of the palmar palpebra, clubbing and skin-limited calcium deposition. Often accompanied by hair, eyebrows, and thin eyelashes. About 25% of patients have malnutrition, and the surface of the nail is rough and hypertrophic, with mediastinum. According to the clinical manifestations, the characteristics of skin lesions and histopathological features can be diagnosed.

Examination of hereditary sclerosing cutaneous heterochromia

Clinical skin examination: The patient's skin lesions are characterized by systemic cutaneous heterochromia, with hyperkeratosis and hardening of the skin at the site of the hair, and other keratosis of the palmar palpebral, clubbing and skin-limited calcium deposition. Often accompanied by hair, eyebrows, and thin eyelashes.

Histopathology: homogenization of collagen fibers and reduction of elastic fibers in dermal tissues.

Other tests: including B-ultrasound, CT, MRI, and chromosomal examination to determine if there are other congenital anomalies.

Diagnosis and diagnosis of hereditary sclerosing cutaneous heterochromia

Should be differentiated from heterochromic dermatitis, vascular atrophic cutaneous heterochromia, and congenital keratosis. The disease should be differentiated from congenital vascular atrophic cutaneous heterochromia, vascular atrophy, skin pigment abnormalities: the disease is an autosomal recessive genetic disease, the main symptoms are skin atrophy, brown red pigmentation, capillaries Expansion, accompanied by congenital cataracts. The two lesions have similarities, but the pathological examination is unreasonable and can be distinguished.

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