Acrokeratosis Paraneoplastica (Bazex Syndrome): A Systematic Review and Quantitative Analysis of 106 Cases
Mustafa Sefa Isin, Mehmet Berke Isler, Amor KhachemouneAbstract
Background
Acrokeratosis paraneoplastica (Bazex syndrome) is an obligate paraneoplastic dermatosis characterized by symmetrical acral psoriasiform lesions and nail dystrophy.
Methods
We conducted a PRISMA-compliant systematic review of 99 publications (89 case reports, 10 case series) describing 106 patients to re-examine the syndrome’s epidemiology, oncologic associations, clinical features, treatment response, and prognosis.
Results
The mean age at onset was 63.5 years (median: 62.5 years; interquartile range (IQR): 57–70 years); men comprised 72.6% (n = 77; 95% CI, 63.5–80.2%), yielding a male-to-female ratio of 2.7:1. Squamous cell carcinoma accounted for 51.9% (55/106; 95% CI, 42.5–61.2%) of associated malignancies, followed by adenocarcinomas (18.9%; 95% CI, 12.6–27.4%) and hematologic neoplasms (8.5%; 95% CI, 4.5–15.4%). Cutaneous lesions preceded the cancer diagnosis in 80.6% (83/103) of patients with reported chronology, with a median lead-time of 5 months (IQR 2–10 months). Nail involvement was documented in 73.6% (78/106). Following oncologic therapy, complete cutaneous clearance occurred in 36.8% (95% CI, 28.2–46.3%) and partial improvement in 30.2% (95% CI, 22.3–39.5%) of cases. In univariable analyses, distant metastasis (p = 0.021) and an aerodigestive primary site (p = 0.026) were nominally associated with reported mortality, although neither remained significant after multiple-comparison correction
Conclusion
Bazex syndrome should be suspected in patients presenting with treatment-resistant acral psoriasiform lesions and nail dystrophy, prompting rigorous malignancy screening.