Inherited ichthyoses
Classifying congenital scaling disorders and recognising systemic risk.
Updated: 2026-09-13
Written for clinicians and medical students
This section is a teaching reference for doctors, residents and medical students. It is not patient guidance and not a substitute for clinical examination, and it must not be used for self treatment.
Clinical framework
- Ask whether scale was present at birth, whether there was a collodion membrane or neonatal erythroderma, and whether hair, eyes, hearing, growth or neurologic development are affected [1].
- Ichthyosis vulgaris usually begins after early infancy with fine extensor scale, palmar hyperlinearity and atopy. X-linked ichthyosis affects boys with larger dark scale and relative flexural sparing [2].
- Autosomal recessive congenital ichthyosis includes harlequin ichthyosis, lamellar ichthyosis and congenital ichthyosiform erythroderma, with overlapping phenotypes requiring molecular testing [1].
Management and escalation
- Neonates with severe ichthyosis require intensive management of temperature, fluids, infection, eye closure, feeding and pain in a humidified environment [1].
- Long-term care combines frequent emollients, bathing and cautious keratolytics. Systemic retinoids may help severe disease but require specialist monitoring and strict pregnancy prevention [1].
- Genetic confirmation supports prognosis, syndrome screening, recurrence counselling and testing of relatives. Check local access to genetic services and do not infer inheritance from appearance alone.