Flaming mother spot on face

Introduction Beckwith-Wiedemann syndrome is an exomphalos-macroglossia-gigantism syndrome, also known as EMG syndrome, Wiedemann type II syndrome, Wilm's tumor and half-body hypertrophy syndrome, Beckwith syndrome, neonatal hypoglycemia visceromegaly macroglossia microcephaly syndrome. According to statistics, the incidence of hypoglycemia accounts for about 33% to 50%. Visceral hypertrophy is also common, such as liver, kidney, pancreas, heart and so on have disproportionate hypertrophy. In addition, there may be face-like flaming mother spots, auricle deformities, and central development of the face.

Cause

Predisposing factors of flaming mother spots on the face

The cause of Bo-Wei syndrome is unknown, and may be an autosomal monogenic hereditary disease. It is also believed that multiple genes may be inherited.

an examination

Related inspection

Whole blood glucose Hb serum glucagon (PG) M-mode echocardiography (ME) two-dimensional echocardiography Doppler echocardiography

Examination of the flaming mother spot on the face

The giant tongue affects the sucking action and causes feeding difficulties, speech disorders, and unclear words. Twitching, giant kidney, giant eyeball, giant ureter, idiopathic cardiac hypertrophy, cryptorchidism, clitoris hypertrophy, diaphragmatic defect, facial erythema, facial flaming mother spot. Easy to suffer from Wilm tumor, hepatoblastoma, nephroblastoma, gonadal tumor, adrenal cancer and so on.

Differential diagnosis

Symptoms of flaming mother spots on the face

1. When the face is affected, the folds deepen to form the "lion face": more common in the primary skin T-cell lymphoma plaque stage, which progresses from the erythema stage, or occurs on normal skin, showing irregular shape and slightly clearer boundaries. The plaques are dark red to purple in color and can be used in varying degrees. It can be self-resolving, or it can be fused to form large plaques. The edges are ring-shaped, bow-shaped or limp-like. When the face is affected, the folds deepen to form the "lion face".

2. Facial congestion is cherry red: due to systemic telangiectasia, increased vascular permeability, can be expressed as facial congestion, facial redness, cherry red, visible in oxide poisoning.

1. Umbilical bulge: It is the main most prominent malformation, generally a giant umbilical bulge, with a base width of more than 6 cm, which may contain the liver and/or small intestine.

2. Giant tongue: The tongue is full of mouth and the tongue is often extended out of the mouth. Because the mouth does not increase, the giant tongue affects the sucking action, which causes feeding difficulties, affecting normal biting, resulting in speech disorder and unclear speech.

3. Giant: The body of the child is significantly larger than the average newborn. The body weight and body length of the giant body are significantly higher than the normal age, and their giant body continues to grow with age. Some manifestations of growth and development, half-length hypertrophy.

4. Hypoglycemia: There is hypoglycemia in the neonatal period, especially in premature infants. Most of the hypoglycemia occurs 24 to 48 hours after birth, even a few hours after birth. Frequent attacks occur in the first month, and severe cases may cause convulsions and loss of consciousness. Hypoglycemia usually stops in 3 to 4 months after birth, and the blood glucose has returned to normal after surgery.

5. Other abnormalities in the umbilicus: There may be umbilical hernia, umbilical cord hernia, and rectus abdominis separation.

6. Other malformations: visceral hypertrophy, giant kidney, giant eyeball, giant ureter, cardiac malformation, idiopathic cardiac hypertrophy, cryptorchidism, clitoris hypertrophy, diaphragmatic defect, microcephaly, poor facial development, earring malformation, facial erythema Face flaming mother spots, etc., especially in the frontal eyebrows.

7. Relationship with tumors: This disease has a tendency to easily affect certain tumors. The literature reports that about 10% of patients have Wilm's tumor, hepatoblastoma, nephroblastoma, gonadal tumor, adrenal cancer and so on. Another report with a half-body hypertrophy is more likely to have a combined tumor, ranging from 25% to 30%.

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