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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
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
• 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
ICHTHYOSES VULGARIS –Small and large scales
attached at centre and turned up at edges
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
X-LINKED ICHTHYOSIS- Smaller, geometric, tightly
adherent, dark brown black scales
• Measures for ichthyoses vulgaris usually suffice,
though need to be used more aggressively

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seminar skin 85.pptx

  • 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