Table 3 Treatment of ichthyosis/MeDOC

From: Inherited ichthyoses/generalized Mendelian disorders of cornification

Topical therapy

 Emollients – non-physiological

1. Occlusive

 Paraffin

 Lanolin

 Non-physiological lipid mixtures

2. Humefectants

 Urea

 Glycerin

 Soaking baths (pure water vs sodium chloride bath additives, oil-based additives and oatmeal-based additives)

 Steam bathsa

 Emollients – physiological

Physiological lipid mixtures (ceramides, fatty acids and cholesterol)

 Keratolytics

Mechanical (eg, pumice stone and scalpel blade)

Salicylic acidb

Alpha-hydroxy acids (lactic-, glycolic acid)

Urea

Soaking baths (pure water vs sodium hydrogen bicarbonate bath additive)

 Disinfectants

 Modifiers of epidermal differentiation

Retinoids

Vitamin D derivativesc

Corticosteroids

Systemic therapy

 Oral retinoids

 RAMBAs

 Antibiotics (in case of severe secondary infections)

  1. Abbreviations: MeDOC, Mendelian disorders of cornification; RAMBAs, retinoic acid metabolism blocking agents.
  2. aBeware of risk of overheating.
  3. bBeware of salicylic acid intoxication if used extensively, in particular in infants.
  4. cConsider monitoring serum calcium to check for possible systemic absorption.
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