Ichthyosis: Drug Therapy

Therapy goals

Hereditary (inherited) ichthyoses: no causal therapy possible → symptomatic therapy!
- Improvement of the skin condition
- Ensure moisture of the skin
- Dissolve cornification and dandruff
- Bring rhagades to heal and avoid new formation
- Prevent infections
- Relief of pruritus (itching)
- Improvement in the quality of life
Acquired ichthyosis:
- Treatment of the underlying disease

Therapy recommendations

Local therapy – “bathe, rub, grease.”
- Hydration of the stratum corneum (horny layer):
  - Balneotherapy (bath therapy) (see below “Further therapy”).
  - Subsequent greasing to maintain the hydration of the skin that has taken place – ointments and creams; suitable are:
    - Dexpanthenol or glycerin-containing products.
    - Water-binding agents such as urea (Urea) – 5-10% in ointment and cream bases.
      - Urea reduces the proliferation (new formation) of the epidermis (epidermis).
      - Urea has a barrier-regenerating, antimicrobial and desquamating effect. The skin is smoothed.
      - Urea should be used in children only from the beginning of the 2nd year of life.
    - In pronounced ichthyosis: 1 to 2 times daily creaming; if necessary, individual areas must be reapplied; in infants and young children may require 6 to 8 times daily creaming.
- Keratolysis (detachment of horn cells) by the following measures:
  - Balneotherapy with keratolytic (desquamation-promoting) bath additives:
    - Sodium hydrogen carbonate (Natriumbicarbonicum pulvis), alternatively baking soda – dosage for adults: 6 g per liter of bath water or 3-4 handfuls per bath tub (∼ 400 g); dosage infants: half the amount of the adult dose (not in the first year of life!).
    - Wheat starch (Amylum tritici)
    - Rice starch (Amylum orycae)
    - Corn starch (Amylum maydis)
  - Keratolytic agents in ointments and creams (as a basis for ointments serve, for example, Eucerin, glycerin, petrolatum, lanolin alcohols):
    - Sodium chloride (table salt), up to 5%.
    - Lactic acid (Acidum lacticum), up to 5%.
    - Polyethylene glycol (Nacrogol 400), 20-30 %.
    - Local retinoids (e.g., vitamin A acid), up to 0.025%; Caveat: Vitamin A acid has the strongest desquamating effect, but quickly leads to skin irritation and burning!
    - Urea (urea), up to 12%.
    - Salicylic acid, despite its good keratolytic properties, should only be used for a short time, if at all, and only on small areas! The acid breaks the disturbed skin barrier and can develop systemic effects as well as acutely toxic (especially in children).
  - Mechanical keratolysis (see below “Further therapy“).
- Notes:
  - If ichthyosis vulgaris is associated with atopic dermatitis, keratolytic therapy must be reduced or interrupted in acute eczema. For hydration, as an alternative to products containing urea, those with polyethylene glycol (20-30%), glycerin (5-20%) or panthenom (5%) can be used.
Systemic treatment (affecting the whole organism).
- In the most severe forms: Acitretin (retinoid = substance related to vitamin A) → inhibits new formation of skin cells and normalizes keratinization, regulates cellular differentiation, increases sweating ability.
- Acitretin is mainly suitable for non-inflammatory ichthyosis: Lamellar ichthyosis, X-linked recessive ichthyosis (XRI), epidermolytic ichthyosis, with limitation also in Comél-Netherton syndrome.
- Cave: do not use in women of childbearing potential! Contraindications and side effects note!
In case of blistering: antiseptic wound treatment.
- Mechanical keratolysis should be done very carefully so as not to encourage the formation of new blisters.
- Prophylaxis: to avoid blisters, the skin should be spared and not exposed to pressure.
For ectropion:
- Eyelid care with products containing panthenol.
- Use of tear substitute fluid
For secondary infections:
- Local antimicrobial treatment
- Systemic antibiotic treatment
In lamellar ichthyosis, Comél-Netherton syndrome, epidermolytic ichthyosis: therapy and prophylaxis with vitamin D preparations.