1, what about pigmentary incontinence?
Mr. yi, a reader from Hebei Province, asked: My child 1 month has pigmented urinary incontinence and now has erythema on his trunk. How to deal with abnormal skin? Should laser be used to remove pigmentation in the future?
Li Zhiming, deputy chief physician of the Department of Dermatology and Sexology, the First Hospital of Peking University, replied: Pigmented urinary incontinence is a genetic disease involving multiple organs, including skin, brain and fundus. According to the skin manifestations of pigmented urinary incontinence, the treatment principles are different in different periods. Erythema blister stage is mainly to protect and prevent infection. Large fused vesicular fluid can be extracted, and astringent drugs such as zinc oxide oil or boric acid solution can be applied externally. If infection is suspected, antibiotic ointment, such as fusidic acid or mupirocin ointment, should be added. In the stage of verrucous hyperplasia, tretinoin ointment can be used externally, and salicylic acid ointment or urea ointment can be used to soften keratin. In the pigmentation stage, although pigmentation seriously affects the appearance, it will mostly fade before puberty to 20 years old, so treatment is not recommended. Laser is not recommended because it is ineffective for pigmentation treatment of pigmented urinary incontinence. ▲
2. Etiology of pigmentary incontinence
1, pathogenesis
It's not clear yet. Some people think that the epidermis can't keep pigment. Pigment cells are inherently permeable or basal cells cannot maintain melanin. The abnormal changes of skin pigment are related to the abnormal configuration of congenital endings in the nervous system. In addition, there are factors such as viral infection and insufficient utilization of congenital vitamins during pregnancy; Opinions vary, and no one can agree.
2. Genetics
According to the investigation of 400 cases of the disease, 55% families have a positive family history, and 32% families have more than two members involved. People have realized that this disease is hereditary. Because the male-female ratio of patients is very different (the male-female ratio is 1: 35), the conclusion of X-linked dominant inheritance is drawn. 50% of the pathogenic genes carried by mothers will be passed on to their children: those with abnormal genes in male fetuses are prone to die in utero; Female fetuses are heterozygotes, and the influence is relatively light, so the birth rate is high, so that most of the cases seen in clinic are women. The expected risks of the offspring of female patients are: female patients, normal baby girls, normal baby boys and aborted male fetuses each account for 1/4.
3. Pathology
A large number of eosinophils can be seen in the blister of erythema, the keratin layer is thickened in verrucous hyperplasia, and there are a large number of melanin in the pigment cells of dermis in papillary hyperplasia and abnormal pigmentation. Cerebral cortex atrophy or cerebellar malformation. The basal cells of bulbar conjunctiva can't retain pigment. Retinal necrosis and nodular hyperplasia of retinal pigment epithelium.
3. Symptoms of pigmentary incontinence
1. Skin symptoms of pigmentary incontinence
It can be roughly divided into four stages: 90% patients develop erythema vesicular inflammation in the early stage around 1-5 weeks after birth, and then turn to the second stage, with papules, pustules and hyperkeratotic stripes, also known as wart stage; In the third stage, dark brown to light gray spiral pigment spots appear on the trunk and limbs, which can also be seen 3-6 months after birth and at the age of one year. The final features include hyperpigmentation and atrophic scar formation.
About 7% patients suffer from unexplained nail malnutrition. Skin symptoms can affect the scalp at all stages. Patients over 1/3 end up bald. In a few cases, some sweat glands are underdeveloped.
2. The nervous system damage of pigmented urinary incontinence.
The following central nervous system symptoms are rare, including seizure (l3.3%), mental retardation (l2.3%), spastic paralysis (1 1.4%) and structural abnormality (7.5%). Long-term follow-up shows that most of the authors who have abnormal central nervous system structure or epilepsy within 4 weeks of birth have serious motor and mental retardation symptoms. Some patients showed brain atrophy or ventricular enlargement after hydrocephalus by pneumoencephalography. Avrahami( 1985) tracked the brain CT changes of a newborn with convulsion. At first, it was focal edema, and after two years, it appeared extensive atrophy. Another 1 1 year-old child has this disease, and the intelligent low brain CT of microcephaly and left spastic hemiplegia is also generalized brain atrophy.
3. Eye signs of pigmentary incontinence
Among the patients, 25-35% have serious eye signs, mostly unilateral, including strabismus, blindness, congenital cataract, microphthalmia, blue sclera, superficial keratitis, iris malformation, vitreous opacity, uveitis, chorioretinitis, nystagmus, myopia, retinal folds or bleeding, optic atrophy and so on.
4. Tooth damage caused by pigmentary incontinence
Two thirds of children over one year old have abnormal teeth. Typical partial or total edentulous crowns are pile-shaped or conical; Because the development or eruption of teeth is blocked, the gap between teeth becomes wider. Permanent teeth are as affected as deciduous teeth. There are also a few reports about high palatal arch and hemorrhagic bone cyst.
Male fetuses usually die in utero, so female patients are more common. It often happens around 1 week after birth. It can be divided into three stages:
(1) Blister stage: Blisters are often arranged in strips, mostly near the trunk and limbs, and may be accompanied by red nodules and plaques, which can last for several days or 1 ~ 2 months. Eosinophils in peripheral blood increased.
(2) Verrucous hyperplasia stage: the skin lesions of hands and feet are red or blue nodules or patches, and they are linear and verrucous hyperplasia.
(3) Pigmentation stage: it is gray-black to brown pigmentation spots with peculiar shapes, such as splash, vortex and line. Pigmentation can last for several years, subside after 10 ~ 20 years old, and the three stages of skin lesions can overlap. Others include pseudoalopecia areata, congenital cataract, epilepsy, mental retardation, tooth deformity and so on.
4. Treatment of pigmentary incontinence
1, no special treatment is required.
At present, there is no specific drug to treat pigmented urinary incontinence, which mainly occurs in baby girls. It usually appears after birth. At first, there will be erythema and blisters on the limbs, then there will be verrucous damage and finally pigmentation. These precipitated pigments will gradually disappear after several years. Usually, pigmented urinary incontinence does not need special treatment, because it will gradually subside and get better after the child is 2 years old, and it will not be noticed in adulthood.
2. Treatment of inflammatory stage
In most cases, it is not necessary to treat pigmented urinary incontinence, as long as the waiting period gradually fades. But some children will have secondary infection and acute inflammation. In the meantime, treatment is needed. Adrenocortin can be used to treat acute inflammation. In addition, if you want to treat pigmented spots, you can use 3% hydroquinone cream externally, or try taking vitamin C orally or intravenously.