All Types of Ichthyosis

Select your type to get your personalised management hub — daily routine, medications, 8-week protocol and more.

Ichthyosis Vulgaris

The most common form

Gene: FLG1 in 250Autosomal DominantEarly childhood onset

Fine white or silvery scaling on limbs and trunk. Often associated with eczema and hay fever. Improves in summer, worsens in winter. Very manageable with consistent emollient routine.

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X-Linked Ichthyosis

Affects males — darker scaling

Gene: STS1 in 2,000–6,000 malesX-Linked RecessiveBirth onset

Dark brown/black scaling on neck and trunk. Affects males; females are carriers. Corneal opacities (harmless). Associated with cryptorchidism in ~25% of cases.

Go to X-Linked Hub →
Lamellar Ichthyosis

Large plate-like scaling, all over

Gene: TGM1 + others1 in 200,000Autosomal RecessiveBirth (collodion)

Born as collodion baby. Large dark plate-like scales over entire body. Ectropion (turned-out eyelids). Reduced sweating — heat intolerance. Requires specialist management and often acitretin.

Go to Lamellar Hub →
Harlequin Ichthyosis

Most severe — improving outcomes

Gene: ABCA121 in 500,000Autosomal RecessiveBirth — NICU

Thick armour-like plating at birth. Diamond-shaped cracking. NICU care essential. Acitretin started immediately. Modern care has dramatically improved survival and quality of life.

Go to Harlequin Hub →
Netherton Syndrome

Severe atopy + bamboo hair

Gene: SPINK51 in 200,000Autosomal RecessiveBirth onset

Trichorrhexis invaginata (bamboo hair). Very high IgE. Severe atopy and anaphylaxis risk. Avoid topical steroids (dangerous absorption). Dupilumab showing good evidence.

Go to Netherton Hub →
KID Syndrome

Keratitis · Ichthyosis · Deafness

Gene: GJB2<200 cases reportedAutosomal DominantBirth onset

Three defining features: vascularising keratitis (eye involvement, can affect vision), hyperkeratotic skin, sensorineural deafness. Increased squamous cell carcinoma risk — lifelong monitoring essential.

Go to KID Hub →
CHILD Syndrome

Always affects one side only

Gene: NSDHL<100 cases reportedX-Linked DominantBirth onset

Congenital Hemidysplasia with Ichthyosiform nevus and Limb Defects. Always unilateral. Metabolic disorder of cholesterol synthesis. Topical cholesterol + lovastatin cream shows dramatic improvement.

Go to CHILD Hub →
PIBIDS Syndrome

Photosensitivity + multiple features

Gene: ERCC2/ERCC3Very rareAutosomal RecessiveBirth/early childhood

DNA repair defect. Photosensitivity, ichthyosis, brittle hair, intellectual disability (variable), decreased fertility, short stature. Extreme sun protection is life-essential.

Go to PIBIDS Hub →
Refsum Disease

Phytanic acid storage disorder

Gene: PHYH / PEX7<200 casesAutosomal RecessiveAdolescence–adulthood

Phytanic acid accumulates causing progressive nerve and organ damage. Ichthyosis is one symptom. Strict dietary restriction (no dairy, ruminant fat) is the primary treatment — not optional.

Go to Refsum Hub →
Self-Healing Collodion Baby

Born collodion — skin clears

Gene: TGM1 + others~10% of collodion babiesAutosomal RecessiveBirth → resolves

Born as collodion baby, skin normalises within weeks to months revealing near-normal skin. Excellent prognosis. May have mild residual dryness. Most go on to live with essentially normal skin.

Go to Self-Healing Hub →
Bathing Suit Ichthyosis

Trunk only — limbs clear

Gene: TGM1 (temp-sensitive)Very rareAutosomal RecessiveBirth onset

Temperature-sensitive TGM1 mutation — enzyme works at lower temperatures, so limbs and face are clear while warmer trunk areas are affected. Management same as Lamellar for affected areas.

Go to Bathing Suit Hub →

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