Patient's question:
Here is the English translation of the provided text:"," translates to:
"All over the body has scaly hard skin, extremely itchy, recently for a 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 decreased number of sebaceous glands. X-linked recessive ichthyosis is characterized by thickening of the stratum corneum and granular layer, prominent acanthosis, evenly distributed lymphocytic infiltration around blood vessels, and a slight decrease in the number of sweat glands. Bullous congenital ichthyosiform erythroderma is characterized by hyperkeratosis and acanthosis, coarse granules in the granular layer, reticular vacuolization in the upper granular and spinous layers, vesicles 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 ichthyosiform 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 pattern, and onset time:
1. Ichthyosis vulgaris: Primarily manifests as dry, brownish rhomboid 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, and the hyperkeratotic scales result from the accumulation of multiple layers of corneocytes that do not shed. Symptoms typically appear several months after birth, worsen around age 5, and may improve after puberty but worsen with age and 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-like scales. The skin is dry and rough, often affecting the entire body, including the axillae and elbow creases; the abdomen and back are particularly affected. 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 and may even worsen.
3. Epidermolytic hyperkeratosis, also known as bullous congenital ichthyosiform erythroderma, is a high-mutation-rate autosomal dominant genetic disease. It is clinically rare. Generalized lesions may appear shortly after birth or within a few months, with thick scales that fall off shortly after birth, leaving widespread erythema and scaling. The erythema may gradually fade, followed by the development of thicker verrucous scales. Localized lesions are limited to the flexor surfaces of limbs and folds, with thick fish-like scales.
4. Lamellar ichthyosis is an autosomal recessive genetic disease. At birth, the entire body is covered by a thick, gauze-like membrane that tightens around the body, often causing eyelid and lip eversion. Within a few days, the membrane falls off, leaving widespread erythema with grayish-white or grayish-brown rhomboid or polygonal scales that are attached at the center and free at the edges. It often appears symmetrically on the entire body, with the most prominent lesions on the flexor surfaces of limbs, elbow creases, axillae, and external genitalia. Hyperkeratosis of the palms and soles, excessive growth 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 and 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 rhomboid or polygonal scales, attached to the skin at the center and free at the edges, with dry skin.
(4) Usually asymptomatic or with only dryness and mild itching.
(5) The condition 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) Lesions commonly occur on the neck, trunk, and flexor folds of limbs, but the palms and soles are unaffected.
(4) The skin is dry and thick, with large, prominent yellowish-brown or brownish-black fish-like scales. The lesions persist throughout life and do not improve or disappear with age.
(5) Corneal opacity often occurs between 20 and 30 years of age.
Lifestyle Advice: 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 survival or lifespan, 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 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 avoid wind and cold exposure, and avoid spicy and irritating foods. This can make patients more comfortable, alleviate symptoms, and help prevent complications. Maintaining a positive mental state is also important.