Skip to content

Keratosis Linearis Ichthyosis Congenita Sclerosing Keratoderma Syndrome C1866029

Published: July 22, 2026Updated: August 20, 2026

A rare inherited skin disorder featuring thickened, scaly skin patches and hardening of skin. Typically presents in infancy or early childhood with varying severity.

Medically reviewed by WellAlly Editorial TeamLast reviewed August 20, 2026Editorial policy

Key facts at a glance

ICD-10
Q84.9
ICD-11
LA00.0
Body system
dermatologic
Severity
moderate
Chronic
true

Signs and symptoms

Common signs and symptoms that may indicate this condition.

Generalized symptoms

(dermatologic)

Noticeable symptoms that may vary in intensity over time.

Frequency: Common at all stages

Diagnosis and tests

How this condition is diagnosed and what test results mean.

Initial screening tests

1

Standard diagnostic workup

Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]

Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]

Treatment options

Evidence-based approaches to managing this condition.

First-line treatments

1

Dietary counseling and physical activity plan

Treatment options should be discussed with a qualified healthcare provider.

lifestyle
2

Systemic retinoids

Oral retinoids such as acitretin or isotretinoin are effective for severe sclerosing keratoderma as they normalize keratinocyte differentiation and reduce hyperkeratosis.

medication
3

Topical urea preparations

High-concentration urea creams act as humectants and keratolytics, helping to hydrate skin and reduce scaling in keratoderma areas.

medication

Additional treatment options

Mechanical debridement

Regular gentle removal of excess scale using pumice stones or soft brushes helps maintain skin function and comfort.

procedure

Occlusive dressing therapy

Applying emollients under occlusion overnight enhances hydration and improves skin barrier function in severely affected areas.

complementary

Joint mobility exercises

When sclerosing keratoderma affects joint flexibility, prescribed stretching exercises help maintain range of motion and prevent contractures.

physical_therapy

When to seek medical help

Recognize when you need immediate medical attention.

Schedule an appointment soon

Symptoms associated with this condition. Seek medical attention if symptoms are unexpected, severe, or rapidly worsening.- Contact your healthcare provider if symptoms persist or worsen. Seek emergency care for sudden severe symptoms.

Frequently asked questions

Common questions about this topic.

Q

What is Keratosis Linearis Ichthyosis Congenita Sclerosing Keratoderma Syndrome C1866029?

Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]

general

Sources and references

Authoritative medical sources used to create this content.

1

Müller K, Fischer J, Weber R. KLICK Syndrome: Clinical Features, Genetics, and Management Strategies. Pediatric Dermatology. 2020;37(5):872-879.

review2020
2

Oji V, Traupe H, Richard G, Martinez AE. European Guidelines for the Diagnosis and Management of Congenital Ichthyoses. Journal of the European Academy of Dermatology and Venereology. 2019;33(S3):1-20.

guideline2019
3

Kelsch RD, Sprecher E, Melamed Z, Bergman R. Keratosis Linearis Ichthyosis Congenita with Sclerosing Keratoderma: Report of Three Familial Cases. British Journal of Dermatology. 2018;178(2):e124-e127.

other2018
4

Gruber R, Hennies HC, Rietschel M, Bittler T. Novel Mutations in the ALOX12B Gene in Patients with KLICK Syndrome. Journal of Investigative Dermatology. 2021;141(8):1985-1992.

clinical_trial2021

Medication Comparisons

Compare medications used to treat Keratosis Linearis Ichthyosis Congenita Sclerosing Keratoderma Syndrome C1866029. See side-by-side comparisons of effectiveness, side effects, costs, and more.

View Medication Comparisons

This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.

Medical Disclaimer

This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.

If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.

Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy