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L11.0 – Acquired Keratosis Follicularis (Darier Disease)

L11.0 is an ICD-10 diagnosis code for acquired keratosis follicularis, also known as Darier disease. A rare chronic skin condition involving abnormal skin keratinization.

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Things worth knowing about "L11.0"

L11.0 is an ICD-10 diagnosis code for acquired keratosis follicularis, also known as Darier disease. A rare chronic skin condition involving abnormal skin keratinization.

What does ICD-10 code L11.0 mean?

The ICD-10 code L11.0 refers to Acquired keratosis follicularis, also known as Darier disease (Darier-White disease). It is a rare, chronic skin disorder characterized by abnormal keratinization of hair follicles and sebaceous glands. The condition belongs to the group of genodermatoses – genetically determined skin diseases – and is inherited in an autosomal dominant pattern.

Causes

The disease is caused by a mutation in the ATP2A2 gene, which encodes a calcium pump (SERCA2) in the endoplasmic reticulum. This mutation leads to impaired cell-to-cell adhesion (acantholysis) and abnormal keratinization (dyskeratosis) of skin cells. The condition typically first presents during puberty or early adulthood.

Symptoms

The typical symptoms associated with L11.0 include:

Small, greasy, scaly yellowish-brown papules (bumps) on the skin, especially in seborrhoeic areas (chest, back, forehead, scalp)
Coalescence of papules into plaque-like lesions with an unpleasant odour
Nail changes: longitudinal ridging, splits, and V-shaped notches at the nail margins
Oral mucosal changes (whitish papules)
Worsening triggered by heat, sweating, UV radiation, and mechanical irritation
Potential psychological distress due to the appearance and odour of lesions

Diagnosis

The diagnosis is usually established through a clinical examination by a dermatologist. A skin biopsy is often performed to confirm the diagnosis, revealing characteristic histological findings (acantholysis, dyskeratosis, corps ronds, and grains). Genetic testing for mutations in the ATP2A2 gene can confirm the diagnosis, although it is not always required.

Treatment

There is currently no cure for Darier disease. Treatment focuses on relieving symptoms and avoiding triggering factors.

General measures

Avoiding heat, sunlight, and tight-fitting clothing
Regular, gentle skin care using emollient preparations
Sun protection (sunscreen, protective clothing)

Pharmacological therapy

Retinoids (e.g., acitretin, isotretinoin): first-line treatment for severe cases; normalize keratinization
Topical keratolytics (e.g., salicylic acid, urea): to soften hyperkeratotic skin areas
Topical or systemic antibiotics/antifungals: in cases of secondary infection
Topical retinoids or corticosteroids: for milder presentations

Surgical and other therapies

Laser therapy or dermabrasion for localized, treatment-resistant lesions

References

Bolognia, J.L., Jorizzo, J.L., Schaffer, J.V. (eds.): Dermatology, 4th edition, Elsevier, 2018.
Cooper, S.M., Burge, S.M.: Darier's disease: epidemiology, pathophysiology, and management. American Journal of Clinical Dermatology, 4(2):97–105, 2003.
World Health Organization (WHO): ICD-10 International Statistical Classification of Diseases and Related Health Problems, 10th Revision, Code L11.0 – Acquired keratosis follicularis.

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