Acrokeratosis paraneoplastica of Bazex

Acrokeratosis paraneoplastica of Bazex (also known as Bazex syndrome) is a rare, paraneoplastic skin disease characterized by psoriasiform changes of the skin, particularly on the acral areas (hands, feet, ears, and nose). The condition is often associated with internal malignancies, most commonly squamous cell carcinomas of the upper aerodigestive tract.

Etiology

The exact cause of Acrokeratosis paraneoplastica of Bazex is unknown. However, it is believed to be a paraneoplastic syndrome, which means it is a disorder triggered by an altered immune system response to a neoplasm (cancer). In most cases, the syndrome is associated with squamous cell carcinomas of the upper aerodigestive tract, including the larynx, pharynx, and esophagus.

Clinical Presentation

Patients with Acrokeratosis paraneoplastica of Bazex typically present with skin lesions that resemble psoriasis. These lesions are often symmetric and are most commonly found on the hands, feet, ears, and nose. Other symptoms may include nail dystrophy, hair loss, and hyperkeratosis of the palms and soles.

Diagnosis

Diagnosis of Acrokeratosis paraneoplastica of Bazex is primarily based on clinical presentation and the presence of an associated malignancy. Skin biopsy may be performed to confirm the diagnosis. The biopsy typically shows psoriasiform hyperplasia, parakeratosis, and a perivascular lymphocytic infiltrate in the dermis.

Treatment

Treatment of Acrokeratosis paraneoplastica of Bazex primarily involves addressing the underlying malignancy. Once the associated cancer is treated, the skin lesions often improve or resolve completely. Topical treatments, such as corticosteroids and retinoids, may be used to manage the skin symptoms.

Prognosis

The prognosis of Acrokeratosis paraneoplastica of Bazex is largely dependent on the stage and type of the associated malignancy. Early detection and treatment of the underlying cancer can significantly improve the prognosis.

See Also

• Paraneoplastic syndromes
• Squamous cell carcinoma
• Psoriasis