Clinical manifestations of congenital ichthyosis

(1) autosomal dominant hereditary unusual ichthyosis,This is a common type of light ichthyosis, substituting one or both parents were home sick more often with catalyst, but no gender differences. Often, a child morbidity, and increased with age, to the most obvious symptoms of adolescence, that is, after the cessation of development. Lesions performance of different degrees of seriousness, the only light to the winter dry skin, no obvious,after powder loading debris, known as dry skin disease. I see hair follicle or in the size of a tip of the hair follicle and brown, without consciously disease, known as hair follicles of ichthyosis (ichthyos; sfoll; cularis). In addition to common skin drying, the idea that or deep brown, central fixation, the free edge. Also was gray and white, with mica-like sheen, gloss of ichthyosis called. More significantly abnormal proliferation of keratinocytes; lcm above can be thick, rough surface, or a spine-like uplift papillary surface dark gray, like bark, herein referred to as superb ichthyosis.
Friends of the disease in the symmetric stretch limbs and trunk side, particularly Zhouxi side for a stretch. Severe cases can be spread to the body, with palmoplantar hyperkeratosis, and that the saddle cleft (toe) A change. Hill like face, scalp, elbow Waterloo, armpit, gills Waterloo, genital, and Tungou often not be violated, or only mildly. Usually without conscious of the winter due to decreased secretion of sweat glands and sebaceous gland, skin drying, and consciously itching discomfort, if the lesion is flu pain. Often patients with atopic constitution, such as fever and asthma subtilis.
Pathology: cuticle significant thickening, particle layer thinning or disappeared. The stratum corneum and stratum corneum cells thickening the desmosome slow degradation, cuticle off the normal cells failed. Spike thin layer cell, basal layer pigment increase. As Hyperkeratosis enter into hair follicle formation of hair follicles Kok suppository. Adenovirus can sweat gland cortical atrophy. No significant changes in the dermis, or in a small number of perivascular lymphocytic infiltration.
(2) of the unusual fish genetic disease: less see. As the disease gene in the X chromosome, is found only in males, may infant after birth or morbidity. Lesions on the type and slightly different large and significant, a tawny or sewage black large, dry rough skin, often around the body, axillary, and the elbow of Waterloo Waterloo, and so can the Department involvement; back especially important than the abdomen . If facial involvement is limited to the ears before and facial aspect. Hair follicle keratosis not normally occur. Palmoplantar from normal skin, skin lesions did not increase with age and reduce, and sometimes it by drama. Female carriers of the disease, does not generally incidence, sometimes only found in the forearm and leg appeared thin. After flexibility and posterior corneal layer membrane turbidity can be a small point, and do not affect vision.
Pathological Hyperkeratosis, normal or granular layer thickness, spine mild thickening layer, the dermis no significant changes can sometimes see in a perivascular lymphocytic infiltration.
Usually seen in clinical samples congenital ichthyosis Erythroderma disease and lamellar ichthyosis as follows:
(C) epidermolysis bullosa of ichthyosis Hyperkeratosis bullous disease, also known as congenital ichthyosis kind Erythroderma disease, with high distortion rate for the autosomal dominant genetic disease. Clinical rare. After birth or after birth a few months, there are generalized and limitations of damage. Pan-onset systemic birth when there is armor-like thick Health after falling out of the emerging pan-sexual flushing, stripping showed moist surface, erythema may gradually disappear, thick wart can happen again -. Only those limitations in the limbs and wrinkle flexor side of the wall will have thick horny tablets. Weight may have defects - like warts hyperplasia or hyperplasia was damage-limitation ask that porcupine-like ichthyosis. The disease started a few years, there will be the small wooden ranging from relaxation of water scars or scars. The use of antibiotics to control infection, and reduce the occurrence of water scars. Palm involvement can affect function.
Pathological epidermal Hyperkeratosis, granular cell layer thickness, the spine of the upper reticular cell vacuolization, only sometimes desmosome associated with the side of spine cell lysis spinous process of epidermal cells, epidermal water scars visible within or scars, a chronic inflammatory dermal shallow infiltration.
(D) lamellar ichthyosis of autosomal recessive genetic, which is a whole body after birth extensive cotton rubbery membrane were tightly wrapped, multi-valgus cause eyelids and lips. A few days later the membrane off, a broad indemnity chronic skin flushing, there are gray or polygonal or rhomboid large, central fixation, the free edge. Often symmetry of the body to physically flexor side, elbow Waterloo, the Waterloo, armpit and genital and other units more distinctly. Palmoplantar hyperkeratosis, fingernails and excessive hair growth. After slow course, there could be life to adulthood erythroderma ease, but still exist. The disease is autosomal recessive inheritance.
Pathology: epidermal middle of Hyperkeratosis, focal keratosis incomplete, granular layer and part of spike cell layer thickening, epidermal sudden extension, a chronic inflammatory dermal shallow infiltration.
2. Treatment:
Yang internal medicine doctor with pure, clean and treatment of ichthyosis, follicular keratosis of, a good effect, the drug from Chinese medicine Astragalus 12 - 18 g. Polygonatum 15 - 18 g. system Shouwu 15 - 30 g. dog Qi 12 -- 18 g. black sesame 12 - 18 g. snake retreat 10 - 15 g. Ling cents 10 - 15 g. Health steroid 18 - 30 g. ginseng 15 - 24 g, and other drug component. skin with moisture. active. flooding Wind the antipruritic effect. with other rub, better efficacy.