Section I. Follicular keratosis
Follicular keratosis, also known as Darier’s disease (DD), is an inherited chronic keratotic skin disease with dyskeratosis of the epidermal cells as the basic pathological change. It is characterized by keratotic and seborrheic papules on seborrheic areas such as the scalp, forehead, behind the ears, chest and back and curved parts of the limbs, and can be accompanied by palmoplantar keratosis and nail damage.
Etiology]
Keratosis pilaris is an autosomal dominant hereditary skin disease with a high ectopic rate, and about 71% of patients have a family history. In addition to genetic factors, some scholars believe that the disease is related to vitamin A metabolism disorder based on the low serum concentration of vitamin A in some patients and the effective application of vitamin A treatment, but the role of vitamin A in the etiology of the disease still needs further study.
Because the early damage is in sun-exposed areas, the lesions worsen after sun exposure and have a seasonal tendency to worsen in summer and improve in winter, it is proposed that sun damage may be an aggravating factor for this disease.
Sometimes the lesions may also occur on infrequently exposed areas such as oral mucosa, groin, palmoplantar and nail bed, and physical friction may also be an aggravating factor because these areas are often susceptible to physical trauma rather than sunlight injury.
Clinical manifestations]
Follicular keratosis usually develops between the ages of 8 and 16 years, and is rare before the age of 5 years, but it can develop at any age. There is no significant gender difference in this disease, and there is a tendency for summer to be heavy and winter to be light.
The lesions are usually found on seborrheic areas such as forehead, scalp, ears, nasolabial folds, neck, shoulders, forehead, mid-back, axillae, etc. They can also be extended to the whole trunk, flexors of limbs, buttocks and external genitalia. The earliest common site of skin lesions is behind the ear. The early lesions are small, firm, normal-skinned papules, mostly symmetrically distributed, occasionally unilaterally distributed, band-like or linear, soon covered with gray, brown or black greasy scabs, after peeling off the scabs, the top of the papules exposed funnel-shaped concave, papules gradually increased into warts, often clustered and tend to fuse, forming irregular warty plaques. The lesions are located in the axillae, groin and other sweaty, friction areas proliferate significantly, often warty or papilloma-like, local moist, with malodor, which is covered with chaps, maceration and purulent exudate.
Diagnosis
According to the clinical manifestations and pathological examination results, the diagnosis is not difficult, but sometimes need to be distinguished from the following diseases.
1, acanthosis nigricans: dark lesions, mostly confined to the axillae, groin and other flexural parts of the body, soft papilloma-like, malignant type often combined with visceral adenocarcinoma.
2.Fused reticulopapillomatosis: It develops in youth, usually between the two breasts and between the scapulae, as yellow-brown flat papules, and gradually fuses into reticulated patches.
3.Seborrheic keratosis: It occurs on the face, back of hands, trunk and upper extremities of middle-aged adults or above, and is a brown flat papule with smooth surface or papilloma-like changes.
Treatment
1. There is no satisfactory treatment. Attention should be paid to avoid hot sun exposure. Vitamin A can be taken on a trial basis, 100-200,000 U per day for at least 2 months, if it is ineffective, then discontinue it, if it is effective, then consider reducing the maintenance. It is important to watch out for vitamin A overdose during treatment, especially in children. Corticosteroids may also be tried, and chloroquine may be tried for photosensitivity. For proliferative rashes with foul-smelling pustules, antibiotic treatment should be given.
2, lesions can be topical keratolytic agents, such as vitamin A acid ointment, sulfosalicylic acid ointment, etc., 5-fluorouracil ointment has some help. For isolated plaque damage, laser, freezing, X-ray irradiation or surgical excision is feasible.
3, Chinese medicine: it is advisable to strengthen the spleen and remove dampness and moisten the skin, and can be used to strengthen the spleen and remove dampness soup, Ermiao Wan, and remove dampness and stomach Ling Tang plus or minus.
Section 2: keratosis pilaris
Keratosis pilaris is a specific keratinization abnormality characterized by the appearance of conical laminae in histology, which are formed by the distribution of thin, cylindrical, closely spaced keratinized imperfect cells throughout the keratin layer. The basic damage is well-defined keratosis imperfecta in the form of a central atrophied ring, linear or patchy.
Clinical manifestations
1, Mibelli type: the damage starts as small, brown, keratotic papules, and gradually expands to form regular and circular plaques with well-defined, raised keratotic borders. The border is often higher than 1 mm and contains linear furrows. The lesions appear hyperkeratotic and verrucous. The center is often atrophic, hairless and sweat-deprived, with hyperpigmentation or loss of pigmentation. There are several lesions ranging from a few millimeters to several centimeters in diameter. They are common on the extremities of the limbs, buttocks, and external genitalia. It is also seen on the face and mouth, and may involve the palmoplantar area.
The type begins in childhood and the lesions expand slowly over several years, often asymmetrically. The type is characteristic, limited, mostly unilateral, with large, well-defined and diagnostic sulci. It is more common in males.
2. Disseminated superficial type and disseminated photodispersive superficial type: DSP is more generalized, mainly involving the extremities, and is bilaterally symmetrical. Damage is also seen in the axillae, groin, perineum, palmoplantar and mucous membranes. In approximately 50% of cases, the damage is seen at the site of exposure (DSAP) and is exacerbated during the summer months. The lesions are small, superficial, relatively homogeneous, and clustered, with even hundreds visible on the extensor surfaces of the extremities. The lesions begin as small keratotic papules, mostly with a small central depression, 1 to 3 mm in diameter, which are mostly dry and may be erythematous, hyperpigmented, or normal in color. DSP and DSAP are relatively common and often develop over several years with a slow onset in the 30s and 40s. Although they are more common in women, there is a 50/50 gender split in family onset. DSAP is seen in areas with strong sunlight and is rare in blacks.
3, palmoplantar disseminated type: small superficial relatively single damage, clear borders, around a dyke-like elevation, the height does not exceed 1 mm. palmoplantar keratinization is more extensive, along the longitudinal grooves of the elevation is more obvious. The lesions are first seen on the palmoplantar area and then spread to the extremities and trunk, including non-exposed areas, in greater numbers. There is pruritus and tingling. The damage on the mucous membranes is small, circular or runny, creamy and asymptomatic, and is more numerous. The incidence is twice as high in males as in females. Onset often begins in adolescence and early adulthood.
4. Linear type: It may appear unilateral, linear and widely involved, similar to linear warty epidermal nevi. The lesions are identical to those of the Mibelli type and include mossy papules, small annular lesions, hyperkeratotic plaques with central atrophy, and characteristic peripheral dyke-like elevations. The lesions are distributed in clusters and arranged in lines along the extremities, with a tendency for distal involvement. A band-like distribution may be seen on the trunk. It may involve the limbs unilaterally, but also the face and trunk ipsilaterally. It begins in infancy or childhood. The genetic characteristics are not clear. It may occur in association with other clinical types. Deterioration has been reported.
5. Mottled type: often associated with Mibelli type or linear type. They are numerous, small, discontinuous spots on the palmoplantar area that are hyperkeratotic with fine raised edges. The lesions may be arranged in a linear pattern or may be aggregated into plaques. Clinical attention must be paid to differentiate it from patchy palmoplantar keratosis.
The diagnosis is generally not difficult based on the clinical presentation and can be confirmed by biopsy if necessary. The histopathological image of the disease has diagnostic value.
Treatment
1, systemic therapy: avoid sun exposure, can be oral chloroquine, 0.25g, 2 times / d. Can try vitamin A 50,000 ~ 100,000 U, 3 times / d; or isotretinoin (13-cis retinoic acid), 0.5 ~ 1mg / kg, 2 times / d.
2, local treatment: the number of small and small range of lesions can be frozen, electrocautery or laser. Topical keratolytic agents, such as 10% salicylic acid ointment or 0.1% retinoic acid ointment, etc.
3, Chinese medicine treatment: the method should nourish the blood and moisten the skin, can moisten the skin pill or warming soup, cangzhi cream.