Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ONGOING

primary (genetic)

1st line – humidified chamber

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ONGOING
|
primary (genetic)
|
collodion baby
1st line – 

humidified chamber

The taut, cellophane-like membrane that may cover newborns with autosomal recessive congenital ichthyosis can be managed with conservative measures, including aggressive hydration with a humidified chamber. This care continues until the membrane is shed. Close monitoring for infection and electrolyte disturbances is important. Ensuring adequate calorie intake is also vital and some babies may require a feeding tube.[24]

Plus – topical emollient

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ONGOING
|
primary (genetic)
|
collodion baby
Plus – 

topical emollient

Treatment recommended for ALL patients in selected patient group

Topical lubrication with petrolatum can enhance the effects of the humidification chamber.[24] However, any type of emollient can be used.

Primary options

petrolatum topical: apply to the affected area(s) twice daily

1st line – increase ambient humidification

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ONGOING
|
primary (genetic)
|
ichthyosis vulgaris or X-linked ichthyosis
1st line – 

increase ambient humidification

Increasing humidity can help to minimize transepidermal water loss. A humidifier placed in the patient's bedroom can alleviate mild cases.

Plus – emollient + humectant + keratolytic

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ONGOING
|
primary (genetic)
|
ichthyosis vulgaris or X-linked ichthyosis
Plus – 

emollient + humectant + keratolytic

Treatment recommended for ALL patients in selected patient group

Plain petrolatum works well as an emollient; however, any type of emollient can be used.

The addition of humectants (hygroscopic compounds) to preparations can be used to increase the water-binding capacity of the skin.

Common humectants include urea and ammonium lactate.

Scaling can be addressed with the application of preparations that contain alpha- and beta-hydroxy acids (e.g., glycolic acid, lactic acid, or salicylic acid), which act as keratolytic agents to break down the scales.[25][26]

Primary options

petrolatum topical: apply to the affected area(s) twice daily

-- AND --

urea topical: (40-50%) apply to the affected area(s) twice daily

or

ammonium lactate topical: (12%) apply to the affected area(s) twice daily

-- AND --

salicylic acid topical: (3-6%) apply to the affected area(s) twice daily

or

lactic acid topical: (10%) apply to the affected area(s) twice daily

or

glycolic acid topical: (4-10%) apply to the affected area(s) twice daily

1st line – emollient + humectant + keratolytic

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ONGOING
|
primary (genetic)
|
autosomal-recessive congenital ichthyosis (lamellar phenotype)
1st line – 

emollient + humectant + keratolytic

The scaling in lamellar phenotype ichthyosis is usually much thicker than that in the primary ichthyoses and may require more aggressive therapy.

In addition to the use of straightforward emollients, the addition of humectants and keratolytics may be required.

Primary options

petrolatum topical: apply to the affected area(s) twice daily

-- AND --

urea topical: (40-50%) apply to the affected area(s) twice daily

or

ammonium lactate topical: (12%) apply to the affected area(s) twice daily

-- AND --

salicylic acid topical: (3-6%) apply to the affected area(s) twice daily

or

lactic acid topical: (10%) apply to the affected area(s) twice daily

or

glycolic acid topical: (4-10%) apply to the affected area(s) twice daily

Consider – topical retinoid

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ONGOING
|
primary (genetic)
|
autosomal-recessive congenital ichthyosis (lamellar phenotype)
Consider – 

topical retinoid

Treatment recommended for SOME patients in selected patient group

Topical retinoids have also been reported as effective for the treatment of lamellar phenotype ichthyosis and can be used in addition to the previously mentioned therapies.[28]

Because irritation is a common adverse effect, topical retinoids are considered as adjunct therapy if moisturizers, humectants, and keratolytics alone are unsuccessful at controlling disease.

Primary options

tretinoin topical: (0.025 to 0.1%) apply to the affected area(s) once daily at night

OR

adapalene topical: (0.1 to 0.3%) apply to the affected area(s) once daily at night

OR

tazarotene topical: (0.05 to 0.1%) apply to the affected area(s) once daily at night

2nd line – oral retinoid

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ONGOING
|
primary (genetic)
|
autosomal-recessive congenital ichthyosis (lamellar phenotype)
2nd line – 

oral retinoid

Oral retinoids are a very effective second-line treatment for lamellar phenotype ichthyosis, and have a long-term adverse-effect profile that is relatively well defined and safe with appropriate monitoring. Treatment with systemic therapy can be long term. If treatment with isotretinoin is being considered as long-term therapy, patients should engage with the iPLEDGE program. iPLEDGE Program Opens in new window

This involves regular monitoring (monthly for 3 months, then quarterly), including CBC, fasting lipids, and liver enzymes.[30]

Oral retinoids should not be used in women who are pregnant or are planning pregnancy because they are teratogenic.

Primary options

acitretin: 25-50 mg orally once daily until improvement, then taper to 10-25 mg two to three times weekly

Plus – emollient + humectant + keratolytic

Back
ONGOING
|
primary (genetic)
|
autosomal-recessive congenital ichthyosis (lamellar phenotype)
Plus – 

emollient + humectant + keratolytic

Treatment recommended for ALL patients in selected patient group

The scaling in lamellar ichthyosis is usually much thicker than that in the primary ichthyoses and may require more aggressive therapy.

In addition to the use of straightforward emollients, the addition of humectants and keratolytics may be required.

Primary options

petrolatum topical: apply to the affected area(s) twice daily

-- AND --

urea topical: (40-50%) apply to the affected area(s) twice daily

or

ammonium lactate topical: (12%) apply to the affected area(s) twice daily

-- AND --

salicylic acid topical: (3-6%) apply to the affected area(s) twice daily

or

lactic acid topical: (10%) apply to the affected area(s) twice daily

or

glycolic acid topical: (4-10%) apply to the affected area(s) twice daily

1st line – emollient

Back
ONGOING
|
primary (genetic)
|
epidermolytic ichthyosis
1st line – 

emollient

Plain petrolatum works well as an emollient; however, any type of emollient can be used.

Primary options

petrolatum topical: apply to the affected area(s) twice daily

Consider – humectant + keratolytic

Back
ONGOING
|
primary (genetic)
|
epidermolytic ichthyosis
Consider – 

humectant + keratolytic

Treatment recommended for SOME patients in selected patient group

Keratolytics and humectants may be added if topical emollients alone do not control disease.[25][26]

Primary options

urea topical: (40-50%) apply to the affected area(s) twice daily

or

ammonium lactate topical: (12%) apply to the affected area(s) twice daily

-- AND --

salicylic acid topical: (3-6%) apply to the affected area(s) twice daily

or

lactic acid topical: (10%) apply to the affected area(s) twice daily

or

glycolic acid topical: (4-10%) apply to the affected area(s) twice daily

Consider – topical antibiotic therapy

Back
ONGOING
|
primary (genetic)
|
epidermolytic ichthyosis
Consider – 

topical antibiotic therapy

Treatment recommended for SOME patients in selected patient group

The corrugated scale that can be present in bioenergetic intolerance elimination (BIE) can create an environment conducive to bacterial overgrowth.

Topical antibiotic therapies can decrease the bacterial growth.

The use of dilute bleach baths, antibacterial soaps, and topical mupirocin can be helpful in treating this if it develops.[32]

Primary options

dilute bleach bath: one quarter cup bleach in a full bathtub once daily

OR

chlorhexidine topical: use to wash body once daily

OR

hexachlorophene soap: use to wash body once daily

OR

mupirocin topical: (2%) apply to the affected area(s) twice daily

2nd line – oral retinoid

Back
ONGOING
|
primary (genetic)
|
epidermolytic ichthyosis
2nd line – 

oral retinoid

Systemic retinoids can also be used as a second-line option, but must be initiated at lower doses with weekly clinical monitoring, as blistering can occur initially in some cases.[25][31] If treatment with isotretinoin is being considered as long-term therapy, patients should engage with the iPLEDGE program. iPLEDGE Program Opens in new window

This involves regular monitoring (monthly for 3 months, then quarterly), including CBC, fasting lipids, and liver enzymes.

Oral retinoids should not be used in women who are pregnant or are planning pregnancy because they are teratogenic.

Primary options

acitretin: 10 mg orally once daily initially, gradually increase according to response, maximum 50 mg /day

Consider – emollient + humectant + keratolytic

Back
ONGOING
|
primary (genetic)
|
epidermolytic ichthyosis
Consider – 

emollient + humectant + keratolytic

Treatment recommended for SOME patients in selected patient group

Plain petrolatum works well as an emollient; however, any type of emollient can be used. Keratolytics and humectants may be added if necessary.[25][26]

Primary options

petrolatum topical: apply to the affected area(s) twice daily

-- AND --

urea topical: (40-50%) apply to the affected area(s) twice daily

or

ammonium lactate topical: (12%) apply to the affected area(s) twice daily

-- AND --

salicylic acid topical: (3-6%) apply to the affected area(s) twice daily

or

lactic acid topical: (10%) apply to the affected area(s) twice daily

or

glycolic acid topical: (4-10%) apply to the affected area(s) twice daily

Consider – topical antibiotic therapy

Back
ONGOING
|
primary (genetic)
|
epidermolytic ichthyosis
Consider – 

topical antibiotic therapy

Treatment recommended for SOME patients in selected patient group

The corrugated scale that can be present in bioenergetic intolerance elimination (BIE) can create an environment conducive to bacterial overgrowth.

Topical antibiotic therapies can decrease the bacterial growth.

The use of dilute bleach baths, antibacterial soaps, and topical mupirocin can be helpful in treating this if it develops.[32]

Primary options

dilute bleach bath: one quarter cup bleach in a full bathtub once daily

OR

chlorhexidine topical: use to wash body once daily

OR

hexachlorophene soap: use to wash body once daily

OR

mupirocin topical: (2%) apply to the affected area(s) twice daily

secondary (acquired)

1st line – modify underlying cause

Back
ONGOING
|
secondary (acquired)
1st line – 

modify underlying cause

Patients with acquired ichthyoses require cessation and/or substitution of any medications that may be causing the condition (e.g., clofazimine, lipid-lowering agents, or butyrophenone antipsychotics), or optimization of management of any underlying conditions such as lymphoma, diabetes mellitus, or systemic lupus erythematosus (SLE).[9][10][11][12]

Plus – emollient

Back
ONGOING
|
secondary (acquired)
Plus – 

emollient

Treatment recommended for ALL patients in selected patient group

Plain petrolatum works well as an emollient; however, any type of emollient can be used.

Primary options

petrolatum topical: apply to the affected area(s) twice daily

Consider – humectant + keratolytic

Back
ONGOING
|
secondary (acquired)
Consider – 

humectant + keratolytic

Treatment recommended for SOME patients in selected patient group

Keratolytics and humectants may be added if topical emollients alone do not control symptoms.

Primary options

urea topical: (40-50%) apply to the affected area(s) twice daily

or

ammonium lactate topical: (12%) apply to the affected area(s) twice daily

-- AND --

salicylic acid topical: (3-6%) apply to the affected area(s) twice daily

or

lactic acid topical: (10%) apply to the affected area(s) twice daily

or

glycolic acid topical: (4-10%) apply to the affected area(s) twice daily

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Choose a patient group to see our recommendations

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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