convoluted linear ichthyosis
Introduction
Introduction to convoluted linear ichthyosis The ichthyosislineariscircumflexa is also known as Netherton syndrome. The health search is an autosomal recessive disorder. The pathogenesis is still not very clear about health search. Health search at birth or shortly after birth, skin lesions for generalized migratory or multi-ring erythema and scaly health search, surrounded by obvious "bilateral" scales, specific. Its generalized erythema and dry and slender scales are like the pathology of psoriasis: showing non-specific changes. Early should be differentiated from ichthyosis-like erythroderma. Multi-ring lesions and hair abnormalities can be identified. 0.1% vitamin A acid cream can be applied externally. The use of corticosteroids can alleviate symptoms. basic knowledge The proportion of the disease: the incidence rate is about 0.001% -0.002% Susceptible people: no specific population Mode of infection: non-infectious Complications: persistent pigmented erythema, psoriasis
Cause
The cause of convoluted linear ichthyosis
Causes:
It is not clear that the ichthyosis linearis circumflexa, also known as Netherton syndrome, is an autosomal recessive disorder. Appears at birth or shortly after birth. Skin lesions are characterized by generalized bilateral or multi-ring erythema and scaly surrounding with distinct "bilateral" scales and specificity. Its generalized erythema and dry and slender scales are like psoriasis.
Pathogenesis:
The pathogenesis is still not very clear.
Prevention
Convoluted linear ichthyosis prevention
Skin care should be strengthened first. When the winter climate is dry, the skin loss is relatively increased, and the skin lesions are aggravated. Therefore, winter is the focus of care, eat more foods rich in vitamin A, such as carrots, butter, sweet potatoes, cod liver oil, chlorophyll and pig liver. It is not advisable to take a bath in winter. Soap should not be used too much. After taking a bath, you should use skin care oil to protect the skin from softness, reduce scales, and maintain proper moisture and adequate nutrients. In addition, you should also pay attention to clothing to keep warm, to avoid cold and irritate the skin; avoid spicy stimulation. This will make the patient comfortable, relieve the condition and prevent it.
Complication
Convoluted linear ichthyosis Complications persistent pigmented erythema psoriasis
Scales, abnormal pigmentation, abnormal hair.
Symptom
Convoluted linear ichthyosis symptoms Common symptoms Hair abnormal squamous pigmented lesions Local migratory erythema
At birth or shortly after birth, the lesions are generalized migratory or multi-ring erythema and scales, with obvious "bilateral" scales around, specific, and its generalized erythema and dry slender The scales are like psoriasis, and the rash on the trunk and the proximal extremities is polymorphous. After the old skin lesions have subsided, new ring lesions can occur in other parts without leaving atrophy, scars or pigments. Calming, skin lesions can be relieved during puberty, but rarely disappear automatically, may have abnormal hair, short hair, crisp, dull, eyebrows, sparse or lack of eyelashes, teeth, nails can be normal.
Examine
Swirling linear ichthyosis
Histopathology: non-specific changes: thickening of the spinous layer, elongation of the epidermis, damage to the marginal keratosis, central hyperkeratosis, thickening of the granular layer and poor keratinization, vasodilation in the dermis,
Diagnosis
Diagnosis and identification of convoluted linear ichthyosis
According to the clinical manifestations, the characteristics of skin lesions and histopathological features can be diagnosed.
Early identification should be differentiated from ichthyosis-like erythroderma, and multiple annular lesions and hair abnormalities can be identified.
