ICTHYOSIS K. Nội TH - 25.03.2014

ICHTHYOSIS
General department





Background
 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.


 The great majority of ichthyoses are inherited, but acquired forms can
develop in the setting of malignancy, autoimmune or infectious disease, and
nutritional deficiency.


Pathophyology
 Clarified by the identification of causative mutations in over 50 genes
encoding structural proteins or enzymes involved in a broad variety of
cellular functions.


 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.

Epidemiology
 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


Physical

 Conjunctiva - Keratinization and thickening secondary to ectropion
 Cornea
 Eyelids
 Retina


Treatment
1.
.
.
2.

.
.
.

Systemic: Oral retinoids :
Etretinate (1 mg/kg/d) (Soriatane)

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


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.


Skin care tips
 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.


Care of neonate
 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


Further Outpatient Care

 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.


Patient Education

 Patients must realize that this condition is chronic, and they will need longterm therapy.

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


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