Keratosis pilaris

Introduction to follicular keratosis Keratosis follicularis, also known as Darier disease, is a rare hereditary disease characterized by poor keratosis of epidermal cells. It usually occurs in the age of 8 to 16 years old. It is rare before 5 years old. There is no obvious difference in race and gender. The condition can be gradually increased with age. The characteristic skin lesions are follicular hard papules with needle tip to pea large, and the top is covered with oily suede. Or scaly scales. basic knowledge The proportion of sickness: 0.1% - 0.2% (in obesity, the incidence of this disease is about 0.1% -0.2%) Susceptible population: 8 to 16 years old Mode of infection: non-infectious Complications: onychomycosis

Causes of hair follicle keratosis

Genetic factors (85%):

The disease is an autosomal dominant genetic disease with complete penetrance, but it also occurs in a single case, possibly due to spontaneous mutation. According to the low serum vitamin A concentration in some patients and the effective use of vitamin A treatment, the disease is considered to be related to vitamin A metabolic disorders.

Environmental factors (15%):

Because early damage is exposed to sunlight, the skin lesions are aggravated after sun exposure, seasonal tendency, summer is aggravated, and winter is improved. Therefore, it is considered that sunlight may be an important pathogenic factor, but skin lesions also occur in non-recurring exposure sites, such as Oral mucosa, groin, palmar and nail bed, because these parts are often subject to physical trauma, so non-sunlight damage may also be a cause of unanimity.

Pathogenesis

The disease is an autosomal dominant genetic disease with complete penetrance. Electron microscopy studies suggest that the primary defects are in the desmosome and tension filaments. Some people are aggravated according to the skin lesions after sun exposure, suggesting that the light damage may also be the cause of the same disease. It may also be associated with vitamin A metabolic disorders.

Hair follicle keratosis 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.

Follicular keratosis complications Complications, onychomycosis

Follicular keratosis can be complicated by hairy infection. Because the pores are blocked, sebaceous gland secretions and sweat can not be discharged. Accumulation in the hair follicle can induce bacterial infection. For patients with low constitution, or long-term use of immunosuppressive agents and fungal infections such as onychomycosis, such as concurrent bacterial infections may have fever, skin swelling, ulceration and purulent secretion.

Symptoms of keratosis of hair follicles Common symptoms: enlarged pores, itchy scaly papules, itchy knots, plantar peeling, purulent discharge, pulmonary fibrosis nodules

It usually occurs in the age of 8 to 16 years old. It is rare before 5 years old. There is no obvious difference in race and gender. The condition can be gradually increased with age. The characteristic skin lesions are follicular hard papules with needle tip to pea large, and the top is covered with oily suede. Or scaly scales, such as the sputum stripping, the funnel-shaped small dimples in the center of the papules, the initial papules are skin-colored, gradually enlarged into irregular plaques, brownish yellow, black or dark brown, often Symmetry occurs in the face, chest, abdomen, extremities, ankles, and the trunk is more damaged in the midline and abdomen. The face is more in the face, the forehead, the ear and the nasolabial fold, and the limbs are more than the flexor, located on the flexor side of the extremities. Under the armpits, the inside of the femoral and other sweats, the damage at the friction is particularly pronounced, often papillary-like, with purulent secretions, stenching stench.

Skin lesions occur in areas of sebum spillage, such as scalp, forehead, ear, nose, neck, chest, scapula, sputum, abdomen, groin, gluteal groove, vulva and limbs. The distribution is often wide and symmetrical, but about In 10% of cases, the distribution of lesions is banded or linear, confined to one side of the body. The scalp damage often covers oily and filth, and generally no hair loss.

About 10% of cases showed apical or diffuse hyperkeratosis in the palm of the hand. The individual papules on the back of the hand and foot resembled the sacral keratosis, and white spots were left after the regression.

A can be affected, manifested as hyperkeratosis, thinning of the deck, brittle fracture, longitudinal white crepe or free margin defect, sometimes nail bed discoloration.

The disease invades the mucosa less, even in the lips, sputum, tongue, buccal mucosa, vagina, esophagus, cecum, white smooth flat or umbilical papules, white plaque or mucosal leukoplakia lesions, occasionally lungs Lesions, mainly in the lower lobe of the lungs, showed nodular shadows and diffuse pulmonary fibrosis on X-ray examination.

Bone cysts, parotid stones, mental retardation and sexual dysfunction are rare.

Generally no subjective symptoms or only mild itching, even itching, damage can be limited for several years or progressive generalized systemic, ultraviolet light can make the skin lesions worse, therefore, the disease is heavy in winter, the general health is very low Affected, a small number of lesions can be secondary to squamous cell carcinoma.

Examination of follicular keratosis

Pulmonary lesions, nodular shadows and diffuse pulmonary fibrosis were seen by X-ray examination. The pathological changes of this disease are not limited to hair follicles. Hairless parts such as palmar and oral mucosa are also affected. Therefore, this disease is not only a disease of hair follicles. The tissue of oral mucosa is similar to that of skin lesions, and there are also dimples and keratosis. However, there is generally no typical round body formation.

Histopathology: The basic changes are poor keratinization, characterized by:

1. There is a round body, grain.

2. The acanthus is loosened above the base layer to form a fissure or a dimple.

3. There are villi (the dermal papilla surrounded by a single layer of basal cells) that protrude regularly into the lacuna.

4. The epidermis can be seen with papilloma with hyperplasia, thickening of the spinous layer, excessive keratinization and embolization of the hair follicle, and chronic inflammation infiltration in the dermis.

The round body is found in the granule or the horn layer, and its center is a condensed nucleus or keratinized substance (large and round, showing a uniform basophilicity), surrounded by a translucent halo, and a basophilic keratosis can be seen around the halo. The substance is like the outer shell. The grain is found in the horns and lacuna. The cytoplasm is rare and the shape is not clear. The nucleus is condensed and often dyed as basophilic. The lacuna is a small fissure-like epidermis blister, usually located at the grass root. Above, where the spiny lysis cells prematurely undergo partial keratinization.

Diagnosis and differentiation of follicular keratosis

diagnosis

According to the brown oily crusting hair follicle papules, it occurs in the sebaceous gland rich and partial, positive family history, with the skin lesions after sun exposure and typical histopathology, the diagnosis is not difficult.

TCM pathogenesis and syndrome differentiation: Chinese medicine believes that the congenital endowment is insufficient, the spleen is not wet, and it is caused by agglutination of the skin. It occurs at an early age and does not cause inflammation. It occurs mostly in wrinkles, and the surface is thick and thick, and the tongue is light. The moss is thin or greasy, and the veins are slow or subtle.

Syndrome: Insufficient congenital, spleen loses health, no moisture, nourish the skin.

Differential diagnosis

Mild cases should be differentiated from seborrheic dermatitis, and localized linear lesions should be identified with sputum.

1. The need to identify the following diseases, the identification points are as follows:

(1) Acanthosis nigricans: manifested as a soft papillary papule, mostly confined to the neck, hernia, groin and other wrinkles, often combined with visceral cancer.

(2) Congenital reticular papillomatosis: The lesion is a flat, large papule, often confined to the upper part of the trunk.

(3) squamous keratosis: often a single sacral nodule of the head or neck.

2. In histopathology should be identified with the following diseases:

(1) Solar keratosis often has an interphase change in the epidermal cell nucleus.

(2) Chronic benign familial pemphigus: no fissures, but a layer of loosened bullae can be seen on the basal layer.

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