ICTHYOSIS K. Nội TH - 25.03.2014

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General department

 A relatively uncommon group of skin

disorders characterized by the presence of excessive amounts of dry surface scales

 A disorder of keratinization or cornification, due to abnormal epidermal differentiation or metabolism

Background

 The great majority of ichthyoses are

inherited, but acquired forms can develop in the setting of malignancy, autoimmune or infectious disease, and nutritional

deficiency

 Clarified by the identification of causative mutations in over 50 genes encoding

structural proteins or enzymes involved in a broad variety of cellular functions

Pathophyology

 Abnormalities in any of these components result in a rather stereotypic epidermal

response with epidermal hyperplasia and

the formation of excess stratum corneum

accompanied by abnormal desquamation

and visible accumulation of scales on the

skin’s surface

 Against environmental allergens, water loss, and infection

 Ichthyosis vulgaris 1/300, X-linked

recessive ichthyosis 1/6000 males

 Mortality/Morbidity: testicular cancer,

cryptorchidism, abnormalities of the sperm count or motility

 Race

 Sex

Epidemiology

 Conjunctiva - Keratinization and thickening secondary to ectropion

 Cornea

 Eyelids

 Retina

Physical

1 Systemic: Oral retinoids :

 Etretinate (1 mg/kg/d) (Soriatane)

 Isotretinoin (2 mg/kg/d) (Amnesteem, Claravis,

Sotret)

2 Ocular:

 Nonpreserved artificial tears

(carboxymethylcellulose sodium 0.5-1.0%) and ointment (white petrolatum 56.8%, mineral oil 41.5%)

 Preservative-free lubricants

 Bandage contact lenses

Treatment

3 Topical :

 Topical cyclosporine A 2% given 3 times

daily

 N-acetylcysteine 10% (Acetadote) emulsion

can be safely and efficaciously used in the topical treatment of neonatal ichthyosis

 Acid salicylic 3%, physiogel, cetaphil,

hydracuta

4 Antibiotics: Erythromycin.

 Apply lotions and creams to wet skin to trap

in the moisture

 Rub gently a pumice stone on wet skin to

help remove thickened crusty skin

 Brush washed hair to remove scales from

scalp

Skin care tips

 Severe congenital ichthyoses:

 Increased transepidermal water loss,

hypernatremic dehydration, and increased heat loss

 Managed in neonatal intensive care units

 Closely monitored for body temperature, vital signs, and electrolytes, infection

Care of neonate

 The mainstay of ichthyosis therapy includes removal of surface scales, and application

of a water barrier

 In disabling cases, oral retinoids may reduce cosmetic disfigurement, depression, and

social isolation

Further Outpatient Care

 Patients must realize that this condition is chronic, and they will need long-term

therapy

 Without long-term therapy, chronic loss of water and calories, which may impair

growth in children

Patient Education

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