Introduction to Albinism

Introduction

PatchyAlbinism(Met disease, white spot disease, piebaldism) is also known as patterned leucoderma (partial albinism). Chinese medicine believes that this disease belongs to the class of white barge. It is only found in the forehead of white forelock, which is rare in clinical practice.

Cause

(1) Causes of the disease

The disease is a congenital leukoplakia without melanocytes, which is autosomal inherited and completely penetrative.

(two) pathogenesis

It is caused by the decreased function of c-kit (mast cell stem cell growth factor), which hinders the differentiation of melanocytes.

symptom

After birth, there are sputum leukoplakia in the anterior temporal region, which is interlaced with black hair. The affected part is white in color, and the eyebrows and eyelashes can also be white. The size and shape of white spots generally do not change with age.

The typical feature of the disease is frontal leukoplakia, 80% to 90% may be associated with forehead white hair, triangular or diamond-shaped, symmetrical, located in the center of the forehead or slightly to one side, can be extended down to the base of the nose. It can also be seen before the squat, the abdomen and abdomen, the upper limbs can see asymmetrical white spots; occasionally there are white spots on the cheeks, the hair at the skin lesions also becomes white, very few patients have only a white hair in the occipital area, and no skin white spots, May be a variant of mottled disease. Some patients have pigmentation spots on the back of their hands and feet. In the center of diffuse leukoplakia, island-like normal skin is visible, while pigmentation around the white spot is thicker. Others may be associated with unilateral iris pigmentation, fundus bleaching and macular dysplasia, strabismus and amblyopia. Some are accompanied by ataxia, deafness and mental retardation, and no self-consciousness.

According to the distribution characteristics of white spots occurring after birth, the diagnosis is not difficult.

diagnosis

Should be identified with vitiligo. Vitiligo is an acquired disease with a late onset, and the damage is not as extensive as the disease, and the pigmentation of the white spot on the edge of the former is more obvious. If there is widening of the eyelid distance, eyelid dysplasia or deafness, Warardenburg syndrome should be considered.

complication

Some are accompanied by ataxia, deafness and mental retardation.

treatment

(a) treatment

There is no special treatment, try PUVA therapy, try melanocyte transplantation and autologous normal pigmented skin grafts.

(two) prognosis

Currently there are no related content description.