atrophic keratosis pilaris
Introduction
Introduction to atrophic keratosis Atrophic keratosis (keratosispilarisatrophicans) is a group of hereditary defects, mainly characterized by keratinization followed by atrophy. It includes three types: facial atrophic keratosis, worm-like skin atrophy, and alopecia keratosis, which can also overlap between types 3. basic knowledge The proportion of illness: 0.002% Susceptible people: infants and young children Mode of infection: non-infectious Complications: neurofibromatosis
Cause
Causes of atrophic keratosis
Genetic (80%):
Facial atrophic keratosis and scarred erythema of the eye are autosomal dominant, and the cause of worm-like skin atrophy is unknown, but there is a clear family predisposition to congenital heart block or other cardiac abnormalities. , neurofibromatosis, mental retardation or Down syndrome.
Pathogenesis
Alopecia keratosis can be X-linked, dominant or autosomal dominant, and an X-linked disease-deficient gene locus has been found to be in Xp22.13~P22.2.
Prevention
Atrophic keratosis prevention
Atrophic keratosis (keratosis pilaris atrophicans) is a group of hereditary defect diseases, mainly characterized by atrophy of keratinization, chromosomal disease can not be treated, and there is no direct prevention. Therefore, the indirect prevention of this disease should be based on early detection, early diagnosis and early treatment. If necessary, chromosome examination during pregnancy can be carried out. For obvious chromosomal abnormalities, termination of pregnancy can be considered.
Complication
Atrophic keratosis complications Complications neurofibromatosis
Insect-like skin atrophy, which may be associated with congenital heart block or other cardiac abnormalities, neurofibromatosis, mental retardation or Down syndrome, and keratosis pilaris atrophicans faciei Noonan syndrome and heart-face-skin syndrome are associated with.
Symptom
Symptoms of atrophic hair keratosis common symptoms palmar keratosis, excessive deafness, repeated infection, blackheads, acne, acne, papules, hair, scarcity, photophobia
1. Facial atrophic keratosis (keratosis pilaris atrophicans faciei) including scarred erythema of the eyebrow and atrophic red keratosis of the hair, more common in young men, generally no scalp scarring alopecia, and Noonan's Syndrome and heart-face-skin syndrome.
(1) erythetic erythema (ulerythema ophryogenes): the damage is persistent reticular erythema and follicular keratotic papules. The middle of the papules has slender eyebrows that are easy to break. Typical damage only affects the outer 1/3 of the eyebrow arch. It can also involve the cheeks, the forehead and the scalp, leaving a point-like depression after regressing, becoming atrophic scar with permanent eyebrows falling off.
(2) keratosis pilaris rubra atrophicans: erythema and follicular papules are symmetrically distributed in the cheeks, sometimes spreading to the forehead, leaving pigmentation, reticular atrophy and scarring.
2. Atrophodic skin atrophy (atrophoder vermiculata) usually occurs in the age of 5 to 12 years old, erythema and needle-sized follicular horn plugs occur in both cheeks and anterior region of the ear, and reticular atrophy rapidly forms after detachment of the angle plug; Limit or extend to the forehead, sputum and ear, the scalp is generally not tired, the typical damage is a myriad of dense worm-like atrophic depressions, about 2mm in diameter, about 1mm in depth, irregular shape, symmetric distribution, between the small concave The narrow, normal skin is separated to give a partial honeycomb and mesh-like appearance; the skin at the atrophy is slightly hard, has a waxy luster, the local color is uneven, and there may be telangiectasia erythema, in which rare blackheads are visible in normal skin. And millet rash-like damage.
Rombo syndrome, or "sand-like skin", refers to worm-like skin atrophy with multiple basal cell carcinoma, hair epithelioma, hair thinning and characteristic hand and foot cyanosis, which is autosomal dominant and needs to be Bazex syndrome, Rasmussen syndrome (milk rash, hair epithelial tumor and cylindrical tumor) and multiple hair epithelial tumors.
3. keratosis pilaris decalvans (keratosis pilaris decalvans) in the onset of infants or childhood, first in the nose, cheeks, filamentous hair follicle horny plug, can be associated with millet rash, keratinous plug off after atrophy Sexual scars, skin lesions can affect the scalp, limbs and trunk, scarring of hair and eyebrows is a feature of this disease, may have excessive palmar keratosis, may be associated with ectopic quality, photophobia, corneal abnormalities, deafness, Physical and mental retardation, repeated infections, abnormalities of the nails and amino aciduria.
Examine
Examination of atrophic keratosis
Histopathology:
1. Facial atrophic keratosis of the hair follicle on the upper 1/3 of the hyperkeratosis, the follicular mouth has a funnel-shaped horny plug, and the pores are dilated, containing one to several curly bristles.
2. The worm-like skin atrophy has different degrees of atrophy, dermal capillaries dilate, vascular wall edema, infiltration of mononuclear cells around blood vessels and hair follicles, hair follicle dilatation, distortion, embolism and keratinous cyst formation, and sebaceous glands are sparse.
3. Hair loss of the hair follicle keratosis, dermis, sebaceous gland atrophy, hair follicle enlargement, with horny plug, occasionally epidermal cyst.
Diagnosis
Diagnosis and differentiation of atrophic keratosis
Diagnosis can generally be made based on clinical manifestations.
However, it needs to be differentiated from small spine moss, red pityriasis, vitamin A deficiency, spastic moss, scar acne, pseudo-alopecia areata and lichen planus.
