[Pathogenesis of acrokeratosis Bazex]

Area of Science:

• Dermatology
• Oncology
• Pathology

Background:

• Acrokeratosis Bazex is a rare paraneoplastic syndrome often associated with squamous cell carcinomas, particularly of the lung and esophagus.
• This case highlights the association between esophageal neoplasm and psoriasiform skin lesions characteristic of Bazex syndrome.

Observation:

• A 67-year-old male presented with psoriasiform skin lesions, a hallmark of acrokeratosis Bazex, nine months post-esophageal cancer diagnosis.
• Clinical examination revealed significant hepatopathy without signs of metastasis.
• Histological, immunofluorescence, and ultrastructural analyses indicated capillaritis with both humoral and cellular inflammation.

Findings:

• Treatment of the esophageal neoplasm led to the regression of the paraneoplastic acrokeratosis.
• Adjunctive topical steroid therapy further aided in the regression of skin lesions.
• Despite treatment, a vitamin A deficiency persisted and could not be corrected through oral or parenteral vitamin A supplementation.

Implications:

• This case underscores the importance of recognizing acrokeratosis Bazex as a potential indicator of underlying malignancy, specifically esophageal cancer.
• The findings suggest that successful treatment of the primary neoplasm is crucial for managing the paraneoplastic syndrome.
• Further research into the pathogenetic mechanisms of Bazex syndrome, including the role of vitamin A metabolism, is warranted.