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Keratosis linearis-ichthyosis congenita-sclerosing keratoderma (KLICK) syndrome is an inherited epidermal disorder characterized by palmoplantar keratoderma, linear hyperkeratotic papules on the flexural side of large joints (cord-like distribution around wrists, in antecubital and popliteal folds), hyperkeratotic plaques (on neck, axillae, elbows, wrists, and knees), mild ichthyosiform scaling, and sclerotic constrictions around fingers that present flexural deformities.
Features include always present findings: Hyperconvex nail. 8 total HPO annotations.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Skin | 6 | Linear arrays of macular hyperkeratoses in flexural areas, Hyperconvex nail, Dry, scaly skin (ichthyosis) |
POMP function has not been fully characterized.
Keratosis linearis-ichthyosis congenita-sclerosing keratoderma syndrome is associated with mutations in the POMP gene on chromosome 13.
Genetic testing for POMP is available. Testing is considered confirmatory for diagnosis.
Phenotype severity distribution: 1 always present feature.
Estimated prevalence: Unknown (Unknown prevalence).
No clinical trials have been registered for keratosis linearis-ichthyosis congenita-sclerosing keratoderma syndrome.
39 publications have been identified in PubMed for keratosis linearis-ichthyosis congenita-sclerosing keratoderma syndrome. Research spans Case Report / Case Series (46%), Review / Meta-Analysis (28%), and Basic Science / Preclinical (13%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 18 | 46% |
Data assembled from 6 of 12 sources · Last updated Sep 20, 2026, 5:35 PM UTC
Online Mendelian Inheritance in Man
European rare disease database
Genetic and Rare Diseases Info Center
1 |
Linear arrays of macular hyperkeratoses in flexural areas |
Pregnancy and birth | 1 | Congenital nonbullous ichthyosiform erythroderma |
Research summaries |
11 |
28% |
Laboratory research | 5 | 13% |
Clinical study results | 2 | 5% |
Disease patterns and progression | 2 | 5% |
Other research | 1 | 3% |
Saleh D (2026). [PMID: 30335335](https://pubmed.ncbi.nlm.nih.gov/30335335/). *Unknown Journal*. [Other]
Cadiravane S (2026). [PMID: 41818120](https://pubmed.ncbi.nlm.nih.gov/41818120/). *J Assoc Physicians India*. [Case Report / Case Series]
Jiang X (2026). [PMID: 41533385](https://pubmed.ncbi.nlm.nih.gov/41533385/). *JAMA Dermatol*. [Case Report / Case Series]
Rossel VSVJ (2026). [PMID: 41781296](https://pubmed.ncbi.nlm.nih.gov/41781296/). *J Dermatol Sci*. [Basic Science / Preclinical]
Nykaza I (2026). [PMID: 41621676](https://pubmed.ncbi.nlm.nih.gov/41621676/). *J Am Acad Dermatol*. [Review / Meta-Analysis]
Rietmann SJ (2026). [PMID: 41601192](https://pubmed.ncbi.nlm.nih.gov/41601192/). *Anim Genet*. [Case Report / Case Series]
Rautiainen N (2026). [PMID: 41074678](https://pubmed.ncbi.nlm.nih.gov/41074678/). *Am J Med Genet A*. [Case Report / Case Series]
Assas M (2026). [PMID: 41914522](https://pubmed.ncbi.nlm.nih.gov/41914522/). *Spec Care Dentist*. [Review / Meta-Analysis]
Nykaza I (2026). [PMID: 41621675](https://pubmed.ncbi.nlm.nih.gov/41621675/). *J Am Acad Dermatol*. [Review / Meta-Analysis]
Metze D (2026). [PMID: 42029647](https://pubmed.ncbi.nlm.nih.gov/42029647/). *Dermatopathology (Basel)*. [Basic Science / Preclinical]