Familial Progressive Pigmentation

Introduction to familial progressive pigmentation Familial progressive hyperpigmentation is an autosomal dominant genetic disease. Pigmentation spots are present after birth. As the age increases, the pigment gradually deepens, the number increases, and the area expands. It is a brown or dark brown pigmentation with inter-island-like normal skin. Invasion of the whole body skin including the palmar, oral and vulvar mucosa and conjunctiva can be involved. Generally, after puberty, the disease develops slowly and gradually, but there is no self-healing tendency. basic knowledge Sickness ratio: 0.0001% Susceptible people: infants and young children Mode of infection: non-infectious Complications: depression

Familial progressive pigmentation

Causes:

Department of autosomal dominant genetic disease.

Pathogenesis:

The pathogenesis is still not clear.

Possible factors:

Genetic factors: Representative diseases include freckles, seborrheic keratosis, neurocutaneous melanosis, pigmented intestinal polyposis, and pigmentation in this group of diseases are localized.

Metabolic factors: hepatolenticular degeneration, hemochromatosis, etc., pigmentation is mostly systemic diffuse, no obvious boundaries.

Endocrine factors: Addison's disease, certain pituitary tumors, longer-term estrogen therapy, pregnancy and chloasma.

Malnutrition: malignant malnutrition, pellagra, vitamin B12 deficiency.

Chemical and pharmaceutical factors: certain anti-tumor and anti-immune drugs, anti-malarial drugs, tar, bitumen, eucalyptus oil and other hydrocarbons.

Physical factors: mechanical trauma, ultraviolet light, certain radiation, chronic itching, scratching and friction.

Inflammation and infectious diseases: chronic inflammation of the skin.

Some tumors.

other factors.

Familial progressive pigmentation prevention

There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease. The cause of this disease is not too clear. It is currently believed that this disease is related to autosomal dominant inheritance. Some scholars believe that it is related to the use of certain drugs and food during pregnancy, so it is temporarily impossible to prevent it. Pregnant women should pay attention to temperament, maintain a happy mood, peace of mind, avoid cold and heat, prevent disease, use drugs with caution, ban tobacco and alcohol, and avoid the effects of poisoning, trauma and physical factors.

Familial progressive pigmentation complications Complications depression

The disease is a benign lesion and generally does not cause damage to other skin or internal organs. However, because the pigmentation of the skin persists, the integrity of the appearance of the skin may be destroyed. Therefore, some people with psychological depression tend to cause psychological damage, and serious cases may cause depression. Therefore, it should cause clinical attention. At present, laser treatment can be used to remove the pigmentation of the skin, but there is a possibility of recurrence.

Familial progressive pigmentation symptoms Common symptoms Pigment pigmentation abnormalities Skin pigmentation deepens

Pigmentation spots are present after birth. As the age increases, the pigment gradually deepens, the number increases, and the area expands. It is a brown or dark brown pigmented spot with inter-island normal skin, which invades the whole body skin including the palmar part, mouth and vulva. Mucosa and conjunctiva can be involved, generally after puberty, the disease develops slowly and gradually, but there is no self-healing tendency.

Familial progressive pigmentation examination

Clinical skin examination: The skin of the disease is pigmented after birth, and it grows progressively with age. It is often brown or dark brown pigmented spots with inter-island normal skin. Invasion of the whole body skin including the palmar, oral and vulvar mucosa and conjunctiva can be involved.

Histopathology: The upper dermal layer of the dermis and the subdermal papillary melanocytes increased. Cell infiltration is often insignificant, with a small amount of lymphocytes and plasma cells sometimes surrounding the blood vessels. The melanin in the lower layer of the epidermis occasionally increased slightly.

Diagnosis of familial progressive pigmentation

diagnosis

Diagnosis can be performed based on clinical performance and laboratory tests.

Differential diagnosis

An exclusionary diagnosis is required, first identifying the two diseases:

1. After scratching or rubbing the skin lesions of pigmented urticaria, the pigment spots are flushed and form a wheal, that is, the Darier sign is positive. In general, the incidence of childhood is mostly, the spot is small, and there may be other damages such as maculopapular rash, papules or nodules. The boundary is clearer.

2. Neurofibromatosis coffee milk spotted torso pigmentation spots vary in size, shape is different, the realm is often clear, visible soft tumors of most prominent protruding surface.

After excluding the above two diseases, further detailed medical history: if there is any history of exposure to chemical substances or physical stimulation, long-term application of certain drugs such as arsenic, silver, chlorpromazine, etc., whether there are endocrine disorders and other medical conditions, such as Addison disease Wait, and conduct the appropriate laboratory tests. It is often necessary to distinguish from drug-induced pigmentation spots, which have a long history of application of certain drugs. Such as silver stagnation, often accompanied by mucosal pigmentation, arsenic is often systemic, especially in the armpits and perineum, often with palmar keratosis.

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