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Also known as:BDCSBazex syndromeBazex syndrome, X-linked dominant
Overview
Bazex-Dupre-Christol syndrome is a rare genodermatosis (hereditary skin disease) with a predisposition to early-onset basal cell carcinomas.
GeneticsX-linked
Clinical Presentation
Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.
Features include very common findings: Coarse hair; and common findings: Milia, Basal cell carcinoma, Sparse hair, and Trichorrhexis nodosa and others. 28 total HPO annotations.
Organ System
Phenotype Count
Example Features
Skin
5
Atopic dermatitis, Hyperpigmentation of the skin, Eczematoid dermatitis
Prognosis
Phenotype severity distribution: 1 very common feature, 9 common features.
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 Bazex-Dupre-Christol syndrome.
10 publications have been identified in PubMed for Bazex-Dupre-Christol syndrome. Research spans Case Report / Case Series (40%), Other (30%), and Review / Meta-Analysis (30%).
Research Type
Count
% of Total
Patient case studies
4
40%
Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 11:56 AM UTC
Diseases are connected by shared genes, phenotypes, therapeutic targets, and MONDO disease hierarchy. The Rare badge reflects Kisho's rarity classification — scored from authoritative sources including OMIM, Orphanet, orphan drug designations, clinical trial activity, and published prevalence data.
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FAQ
Frequently Asked Questions
Common questions about Bazex-Dupre-Christol syndrome
Bazex-Dupre-Christol syndrome is a rare inherited skin condition. It is known as a genodermatosis, meaning it primarily affects the skin. People with this condition have a predisposition to developing basal cell carcinomas at an early age.
This syndrome is recognized as a hereditary disease, and its features have been noted in several medical resources. While the core feature involves skin tumors, other skin abnormalities may also be a part of the condition.
Information about symptoms is currently limited for this condition. However, it is noted that early onset basal cell carcinomas is a characteristic feature, and the condition name suggests that there may be skin changes such as follicular atrophoderma and other skin abnormalities.
Bazex-Dupre-Christol syndrome is caused by genetic changes. It is linked to a mutation in the BZX gene and is inherited in an X-linked dominant manner. This means that the condition can be passed from one generation to the next through the genetic information on the X chromosome.
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