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Living with Autosomal recessive congenital ichthyosis 11

An inherited skin condition that causes scaling over the whole body and sparse hair, starting at birth or in infancy.

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Understanding Autosomal recessive congenital ichthyosis 11

Autosomal recessive congenital ichthyosis 11 is an inherited skin condition that causes scaling over the whole body.

Autosomal recessive congenital ichthyosis (ARCI) is a group of disorders that are limited to the skin. The two main skin patterns are lamellar ichthyosis, with large, dark scales and no redness, and a type with redness and fine white scales. Signs start in the newborn period or infancy. Many babies are born with a tight, shiny covering on the skin (collodion membrane).

Signs & symptoms

Symptoms vary from person to person. GARD lists these among the most common:

  • Scaly skin (ichthyosis)
  • Sparse hair

Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.

Diagnosis

  1. Talk with your doctor

    Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.

  2. See a specialist

    Specialists who may be involved include genetic specialists and skin, hair, and nail specialists.

  3. Consider genetic testing

    Changes in ST14 are linked to this condition. Genetic testing and counseling can help confirm a diagnosis and explain what it means for your family.

  4. Connect with expert centers

    Foundation for Ichthyosis and Related Skin Types may keep lists of experts for this condition.

Care & treatment

Care team
Genetic specialists (geneticists); Skin, hair, and nail specialists (dermatologists)
Expert care
Expert lists: Foundation for Ichthyosis and Related Skin Types
Treatment
GARD doesn't list specific treatments for this condition. Your care team will tailor care to your symptoms.
Research
Clinical trials and observational studies may be options. See the research section below.

General information, not medical advice. Your care team makes the decisions.

Everyday life

Living with a rare condition is easier with support.

  • Connect with Foundation for Ichthyosis and Related Skin Types and other patient organizations
  • Keep notes on symptoms, changes and questions for appointments
  • Ask about patient registries that help research
  • Look into financial aid and travel resources for care visits
Bring these questions to your next doctor visit
Can genetic testing for the ST14 gene confirm the diagnosis?
Which type of ichthyosis scaling do I or my child have?
What skin care routine works best for scaling and cracks?

Quick facts

Symptoms usually startNewborn and as an Infant
InheritanceAutosomal Recessive
GenesST14

It is caused by changes in the ST14 gene. It is autosomal recessive, so a child inherits a changed copy of the gene from each parent.

GeneticSkin

Also known as: autosomal recessive congenital ichthyosis type 11; autosomal recessive ichthyosis with hypotrichosis; hypotrichosis-congenital ichthyosis syndrome; ichthyosis and follicular atrophoderma with hypotrichosis and hypohidrosis; ichthyosis-follicular atrophoderma-hypotrichosis syndrome; ichthyosis-follicular atrophoderma-hypotrichosis-hypohidrosis syndrome

Patient organizations

Source: NIH GARD · Autosomal recessive congenital ichthyosis 11GARD ID 10116 · GARD page updated September 2026 · Plain-language summary by Know Rare; editorial review pending. General information, not medical advice.

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