Patient's question:
Here is the English translation of the provided text:"," translates to:
"All over the body there is scaly hard skin, extremely itchy, for the past week"
Doctor's answer:
Ichthyosis vulgaris is characterized by thinning of the epidermis, mild to moderate thickening of the stratum corneum, reduced or absent granular layer, keratin plugs in hair follicles and sweat glands, and a decrease in the number of sebaceous glands. X-linked recessive ichthyosis is characterized by thickening of the stratum corneum and granular layer, prominent rete ridges, evenly distributed lymphocytic infiltration around blood vessels, and a slight decrease in the number of sweat glands. Bullous congenital ichthyosis-like erythroderma is characterized by hyperkeratosis and acanthosis, coarse granules in the granular layer, reticular vacuolization in the upper granular and spinous layers, blisters within the epidermis, and mild inflammation in the superficial dermis. Lamellar ichthyosis is characterized by moderate hyperkeratosis, focal hypokeratosis in some cases, thinning or mild thickening of the granular layer, moderate acanthosis, and inflammatory cell infiltration in the upper dermis. Non-bullous congenital ichthyosis-like erythroderma is characterized by hyperkeratosis, mild hypokeratosis, and acanthosis, with lymphocytic infiltration in the superficial dermis.I. Types of Ichthyosis: Clinically, ichthyosis is divided into four types based on symptoms, inheritance patterns, and onset time:
1. Ichthyosis vulgaris: Primarily manifests as dry, brown, rhombic or polygonal scales on the extensor surfaces of limbs and trunk. Prominent keratotic papules are often seen on the extensor surfaces of the arms and thighs, and the palms and soles are affected. The incidence is high, with hyperkeratotic scales formed by the accumulation of multiple layers of corneocytes that do not shed. It typically appears several months after birth, with the most severe symptoms around age 5. Symptoms may improve after puberty but worsen with age due to improper treatment.
2. X-linked recessive ichthyosis: Can develop after birth or in infancy. The scales are large and prominent, appearing as large, yellowish-brown or dirty-black fish-scale-like patches, with dry, rough skin often covering the entire body. The axillae and elbow creases may also be affected, with the abdomen and back being particularly severe. If the face is affected, it is limited to the preauricular and lateral facial areas. Follicular keratosis does not typically occur. The skin of the palms and soles is normal, and the lesions do not improve with age but may worsen. Corneal opacity often occurs between the ages of 20–30.
3. Epidermolytic hyperkeratosis, also known as bullous congenital ichthyosis-like erythroderma, is a high-mutation rate autosomal dominant genetic disease. It is rare clinically. Generalized or localized lesions may appear shortly after birth. In generalized cases, the entire body is covered with thick scales at birth, which fall off shortly after birth, revealing widespread erythema and scaling. The erythema may gradually fade, followed by the development of thicker verrucous scales. In localized cases, thick fish-scale-like hyperkeratosis is only present on the flexor surfaces of limbs and flexural folds.
4. Lamellar ichthyosis: An autosomal recessive genetic disease. At birth, the entire body is covered with a wide, thick layer of cotton-candy-like membrane, often causing ectropion of the eyelids and everted lips. After a few days, the membrane falls off, leaving widespread erythema with grayish-white or grayish-brown rhombic or polygonal scales that are attached at the center and free at the edges. It is often symmetrical and affects the entire body, with the flexor surfaces of limbs, elbow creases, axillae, and external genitalia being particularly prominent. Hyperkeratosis of the palms and soles, overgrowth of nails and hair, and a slow course of the disease are observed. The erythroderma may improve in adulthood, but the scales persist.
Guidelines: Ichthyosis is generally classified as autosomal dominant ichthyosis vulgaris and X-linked ichthyosis.
I. Autosomal dominant ichthyosis vulgaris:
(1) Typically develops between 1–4 years of age, affecting both males and females.
(2) Lesions commonly occur on the extensor surfaces of limbs and trunk.
(3) The lesions appear as light brown to dark brown rhombic or polygonal scales, attached centrally to the skin and free at the periphery, with dry skin.
(4) Usually asymptomatic or with only mild dryness and itching.
(5) The skin worsens in winter and improves in summer. Symptoms and signs may lessen or recur after adulthood.
(6) Often accompanied by hyperkeratosis of the palms and soles, fissures, and changes in nails.
II. X-linked ichthyosis:
(1) Typically develops within 3 months after birth, usually not exceeding one year.
(2) This type of ichthyosis is almost exclusively seen in males, with females being carriers.
(3) The skin is commonly affected on the neck, trunk, and flexural folds of limbs, but the palms and soles are spared.
(4) The skin is dry and thick, with large, prominent, yellowish-brown or brownish-black fish-scale-like patches. The lesions persist throughout life and do not improve or disappear with age.
(5) Corneal opacity often occurs between the ages of 20–30.
Lifestyle Care: Ichthyosis is a genetic disease with its root in genes. Currently, it cannot be cured, and treatment cannot alter the inherited genes. Therefore, treatment does not affect the genetic probability. Ichthyosis does not harm health and does not affect the survival or lifespan of patients, but it can impact appearance and mental well-being. Medically, ichthyosis is not a disease that disqualifies individuals from marriage, and patients can marry and have children. Different types of ichthyosis have different modes of inheritance and probabilities.
Eat more foods rich in vitamin A, such as carrots, cream, sweet potatoes, cod liver oil, green leafy vegetables, and pork liver. Avoid excessive bathing in winter, use minimal soap, and apply moisturizing oils after bathing to keep the skin soft, reduce scaling, and maintain proper hydration and nutrients. Additionally, wear warm clothing to protect the skin from wind and cold, and avoid spicy and irritating foods. This can help patients feel comfortable, alleviate symptoms, and achieve preventive effects. Maintaining a positive mental state is also important.