follicular ichthyosis
Introduction
Introduction to hair follicle ichthyosis Follicularisichthyosis, also known as keratosispilarisdecalvans, is unknown. The pathogenesis is still unclear. The clinical manifestations are systemic hyperkeratosis, accompanied by flesh-colored and thorn-like generalized hair follicle bulge, which occurs in the baby's face. The skin lesions are characterized by miliary rash, central horned plugs, and some are spinous, forming later. The skin is atrophied. Also known as alopecia spine keratosis, Siemen syndrome. The systemic symptoms vary greatly. The ratio of male to female incidence is 5:1. basic knowledge The proportion of illness: 0.005% Susceptible people: infants and young children Mode of infection: non-infectious Complications: swelling
Cause
Causes of hair follicle ichthyosis
(1) Causes of the disease
The etiology of this disease is not clear, and may have certain correlation with environmental factors, genetic factors, dietary factors, and mood and nutrition during pregnancy.
(two) pathogenesis
The pathogenesis is still unclear. Histology may have hair follicle mouth due to the presence of angle plugs, causing the abdomen of the hair follicle to expand, sometimes with curled hair in the lower third of the area. Moderate keratosis is accompanied by thinning of the granular layer.
Prevention
Hair follicle ichthyosis prevention
Early detection, early diagnosis, and early treatment are important for indirect prevention of this disease, and can also reduce the incidence of infection complications. For patients with existing infections, antibiotics should be used as soon as possible.
Complication
Follicular ichthyosis complications Complications swelling
Because the disease may be accompanied by itching and the integrity of the skin is destroyed, it may cause skin bacterial infection or fungal infection due to scratching, usually secondary to low constitution, or long-term use of immunosuppressants and fungal infections such as onychomycosis. Such as concurrent bacterial infections may have fever, skin swelling, ulceration and purulent secretion and other performance. Severe cases can lead to sepsis, which should be brought to the attention of clinicians.
Symptom
Hair follicle ichthyosis symptoms common symptoms photophobia layered ichthyosis deafness visual acuity angle plug
Occurs in the baby's face, the skin lesions are miliary rash, the center has a horned plug, and some have a spinous shape. After the formation, the skin is atrophied. In addition to the face and cheeks, it can also be seen in the neck and limbs, which occurs in the eyelids. Department and scalp, after the formation of alopecia scars, some patients are limited to the rash of the palmar palpebral, some patients may be associated with corneal thin, visual impairment, photophobia, deafness and poor sweating.
Examine
Examination of hair follicle ichthyosis
Clinical skin examination: The skin lesions are characterized by miliary rash, central horn plugs, and some are spinous, and the skin is atrophied. In addition to the face and cheeks, it can also be found in the neck and limbs. Occurred in the eyelids and scalp, after the formation of alopecia scars. Some patients are limited to rashes in the palm of the hand.
Histopathology: The hair follicle mouth is caused by the angle plug, which causes the abdomen of the hair follicle to expand, sometimes with curled hair in the lower third part. Moderate keratosis is accompanied by thinning of the granular layer. Late connective tissue atrophy forms scars.
Diagnosis
Diagnosis and identification of hair follicle ichthyosis
diagnosis
According to the clinical manifestations, the characteristics of skin lesions and histopathological features can be diagnosed.
Differential diagnosis
The disease is differentiated from infant miliary rash and impetigo, and its clinical manifestations are similar to those of follicular ichthyosis, but the pathological mechanism is different.
1. Miliary rash: common in the rubbing area, the rash is the size of the cap needle white dense thin wall transparent small blisters, about 1mm size, the blister wall is easy to break, the appearance is dew-like, there is no redness around, it appears quickly, and quickly dry after a few days And disappeared, after drying, there are very thin and small scales, which naturally fall off. Often have no symptoms. More common in bedridden, high fever and poor sweat and postoperative infirm, vitamin A deficiency caused by this type of reduction.
2. Impetigo: is a common acute suppurative skin disease. It has the characteristics of contact infection and autologous infection, and is easy to be popular among children. The pathogens are mainly coagulated plum-positive Staphylococcus aureus or beta-hemolytic streptococcus alone or in combination.
