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hypotrichosis-osteolysis-periodontitis-palmoplantar keratoderma syndrome - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

hypotrichosis-osteolysis-periodontitis-palmoplantar keratoderma syndrome

MONDO:0011884

Also known as:HOPP syndromehypotrichosis-osteolysis-periodontitis-palmoplantar hyperkeratosis syndromehypotrichosis-striate palmoplantar hyperkeratosis-acroosteolysis-periodontitis syndrome

Overview

Hypotrichosis-osteolysis-periodontitis-palmoplantar keratoderma syndrome is an extremely rare ectodermal dysplasia syndrome characterized by hypotrichosis universalis with mild to severe scarring alopecia, acro-osteolysis, onychogryphosis, thin and tapered fingertips, periodontitis and caries leading to premature teeth loss, linear or reticular palmoplantar keratoderma and erythematous, scaling, psoriasis-like skin lesions on arms and legs. Lingua plicata and ventricular tachycardia have also been observed.

Diagnosis

Biomarker and diagnostic research for hypotrichosis-osteolysis-periodontitis-palmoplantar keratoderma syndrome has been reported in the published literature.

Prognosis

Estimated prevalence: <1 in 1,000,000 (VERY_RARE).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for hypotrichosis-osteolysis-periodontitis-palmoplantar keratoderma syndrome.

248 publications have been identified in PubMed for hypotrichosis-osteolysis-periodontitis-palmoplantar keratoderma syndrome. Kisho has analyzed 50 by research type. Research spans Review / Meta-Analysis (38%), Case Report / Case Series (28%), and Basic Science / Preclinical (12%).

Research Type	Count	% of Total
Research summaries	19

Data assembled from 4 of 12 sources · Last updated Sep 20, 2026, 5:36 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:607658

Orphanet

European rare disease database

Orphanet:307936

GARD

Genetic and Rare Diseases Info Center

GARD:17384

Questions for Your Care Team

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hypotrichosis-osteolysis-periodontitis-palmoplantar keratoderma syndrome

Overview

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

OMIM

Orphanet

GARD

Related Diseases

Questions for Your Care Team

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