Progressive acromelanosis

Introduction to progressive acromegaly Progressive acromosis (progressive acromosis), also known as progressive acral pigmentation, is a progressive acral pigmentation disorder characterized by diffuse progressive melanin deposition on the dorsal side of the toe. Have a family history. basic knowledge The proportion of illness: 0.03% Susceptible people: no specific population Mode of transmission: mother-to-child transmission Complications: hereditary symmetrical pigment abnormalities

Causes of progressive acromegaly

Causes:

The cause is unknown, mostly autosomal dominant inheritance, which may be related to the marriage of a close relative.

Pathogenesis:

The pathogenesis is still inaccurate and may be autosomal dominant.

Progressive acromegaly prevention

Avoid close relatives getting married.

Complications of progressive acromegaly Complications, hereditary symmetry, pigmented abnormalities

The pigment is abnormal and the skin is metallic or slate gray.

Symptoms of progressive acromegaly common symptoms mental retardation epilepsy and epileptic seizures a large number of skin desquamation

2 to 6 months after birth, there is a limited melanin deposition on the finger (toe) and the dorsal side of the finger. The boundary is obvious, the surface is smooth, there is no scaling, and the nail is not invaded. After 4 years old, the skin lesion develops progressively, and the groin In the lower abdomen, the femur, the buttocks, the lower extremities, the vulva, the armpits and other parts may appear hyperpigmentation, the child's mental retardation may have epileptic seizures, and some may be accompanied by pigmented nevus and edgy.

Examination of progressive acromegaly

Histopathology: increased melanin at the junction of dermis and epidermis, mild hyperkeratosis of the epidermis, melanosomes in the stratum corneum, a small number of melanocytes in the superficial dermis, and mild perivascular infiltration.

Diagnosis and differentiation of progressive acromegaly

According to the clinical manifestations, the characteristics of skin lesions and histopathological features can be diagnosed.

It should be differentiated from Kitamura reticular acral pigmentation.

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