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Ichthyoses are a group of skin disorders characterized by dry, thickened, fish-like scales on the skin that worsen in winter. There are several types that are usually inherited but some may be acquired. Ichthyosis vulgaris has an autosomal semi-dominant inheritance pattern linked to a filaggrin gene mutation, causing reduced filaggrin and small or large scales on the shins. Treatment focuses on moisturizing the skin through hydration, emollients, and moisturizers. X-linked ichthyosis has an X-linked recessive pattern due to steroid sulfatase deficiency, with large, dark geometric scales on the shins and smaller scales elsewhere. Genetic counseling is
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- The document discusses capacity building, grievance redressal, awards for best performing Rural Kevel Swasthya Samitis (RKS), and patients' rights for members of RKS. It also discusses establishing a purchase committee to oversee procurement using RKS funds.
- Key aspects include organizing continuous training and orientation programs for RKS members, establishing a grievance redressal system, assessing RKS performance to provide awards, and outlining several patients' rights including access to care, informed consent, and right to complain.
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STRIDOR .pptx
Stridor refers to an abnormal high-pitched breathing sound caused by partially obstructed airflow in the airway. It can be inspiratory, expiratory, or biphasic depending on the location of obstruction. Common causes of stridor in children include laryngomalacia, vocal cord paralysis, croup, and foreign body aspiration. Evaluation involves history, examination, and may include laryngoscopy, imaging, and other tests to determine the specific cause. Management is directed at treating the underlying cause through medications, surgery, or removing any obstructions.
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- 1. DISORDERS OF KERATINIZATION ICHTHYOSES Heterogeneous group of dermatoses characterized by presence of fish-like scales, with scaling generally worse in winters. Usually inherited, but may be acquired. TYPES 1. ICHTHYOSES VULGARIS 2. X-LINKED ICHTHYOSES 3. LAMELLAR ICHTHYOSES 4. NONBULLOUS ICHTYOSIFORM ERYTHRODERMA 5. EPIDERMOLYTIC ICHTHYOSES
- 2. ICHTHYOSES VULGARIS ETIOLOGY • Inheritance – has a autosomal semi dominant inheritance • Molecular defect- Filaggrin gene resulting in reduced filaggrin in skin CLINICAL FEATURES • Symptoms- usually asymptomatic or may have mild itching , especially on legs in winter CHARACTER OF SCALES • On shins- Most severely affected , generally small, sometime large scales attached at centre and turned up at edges , making the skin feel rough
- 3. • If severe patient may develop superficial fissuring on shins in winters TREATMENT • Restricting hot water baths and cleansing lotions • Hydration of skin by soaking in bath tub • Use of emollients including vegetable oil and mineral oil • Use of moisturizers like glycerol
- 4. ICHTHYOSES VULGARIS –Small and large scales attached at centre and turned up at edges
- 5. X-LINKED ICHTHYOSIS ETIOLOGY • Inheritance- x-linked recessive inheritance • Molecular defect- deficiency of enzyme steroid sulfatase CHARACTER OF SCALES • Shin-Large, geometrical, tightly adherent, dark brown-black scales • Rest of body- Smaller, but similar scales TREATMENT • Genetic counselling • Oral retinoids best avoided, due to side effects and benign nature of disease
- 6. X-LINKED ICHTHYOSIS- Smaller, geometric, tightly adherent, dark brown black scales • Measures for ichthyoses vulgaris usually suffice, though need to be used more aggressively