Ichthyosis is a common hereditary skin keratinization disorder, formerly known as ichthyosis vulgaris, referred to as "ichthyosis" in Chinese medicine. It primarily manifests as dry, rough skin on the extensor surfaces of the limbs or the trunk, accompanied by rhomboid or polygonal scales resembling fish scales or snake skin. Depending on the inheritance pattern, it can be classified into autosomal dominant ichthyosis vulgaris, X-linked ichthyosis vulgaris, congenital ichthyosiform erythroderma, and lamellar ichthyosis.

bubble_chart Pathological Changes

1. Dominant hereditary ichthyosis: Grade II hyperkeratosis of the epidermis, accompanied by thinning or disappearance of the granular layer;
2. X-linked ichthyosis vulgaris: Hyperkeratosis with normal or slightly thickened granular layer;
3. Epidermolytic hyperkeratosis ichthyosis: Hyperkeratosis, epidermal cell lysis, and granular degeneration.

bubble_chart Type

According to symptoms and signs, it can be divided into four types:

1. Dominant ichthyosis vulgaris
   1. Onset occurs between 3 months and 5 years after birth;
   2. Dry skin covered with gray-white to light brown water calptrop base-shaped or polygonal scales, with raised edges and the center tightly adhered to the skin;
   3. Mainly distributed on the extensor surfaces of the limbs and the back;
2. X-linked ichthyosis vulgaris
   1. Onset occurs within 3 months after birth, affecting only males;
   2. The skin is dry and thickened, covered with large, dark brown scales, worsening in winter and improving in summer. With age, the lesions remain unchanged or even worsen;
   3. Skin lesions are distributed all over the body, with the most severe involvement on the head, face, sides, front of the ears, and neck. Flexural areas are often affected.
3. Congenital ichthyosiform erythroderma
   1. At birth, the entire body is covered with armor-like multilayered scales, which shed to reveal rough, moist surfaces, sometimes with loose blisters;
   2. In severe cases, the hands and feet become claw-shaped;
   3. Autosomal dominant inheritance.
4. Lamellar ichthyosis
   1. At birth, the infant's entire body is tightly wrapped in a widespread collodion-like membrane, which sheds after a few days, leaving the skin extensively reddened with large polygonal scales, the center adhering and the edges free;
   2. Hyperkeratosis of the palms and soles, with overgrowth of nails and hair;
   3. Two-thirds of patients have ectropion.

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bubble_chart Treatment Measures

1. There is currently no cure, and the goal of treatment is to relieve symptoms, increase stratum corneum hydration, and promote normal keratinization;
2. Systemic treatment may include trials of vitamin A, 13-cis retinoic acid, etretinate, or methotrexate;
3. Topical treatments can be used to increase stratum corneum hydration and remove excessive keratinization, such as urea cream, retinoic acid, salicylic acid, etc.;
4. For infections, topical antibiotics ointment may be applied.

bubble_chart Cure Criteria

1. Cured: Skin lesions subside by more than 90%;
2. Improved: Skin lesions subside by more than 50%;
3. Not cured: Skin lesions subside by less than 30%.