Bazex syndrome

Bazex Syndrome, also known as Acrokeratosis Paraneoplastica, is a rare paraneoplastic syndrome associated primarily with squamous cell carcinomas of the upper aerodigestive tract. First described by Bazex et al. in 1965, this condition is characterized by the development of psoriasiform plaques on the acral parts of the body, notably the hands, feet, nose, and ears. It is considered a cutaneous marker for malignancy, with the skin manifestations often preceding the diagnosis of the underlying tumor.

Symptoms and Signs

The hallmark of Bazex Syndrome is the appearance of scaly, psoriasis-like plaques that typically begin on the ears, nose, hands, and feet. These lesions can spread to involve the nails, causing dystrophic changes, and may also affect the knees and elbows. Patients may experience pruritus (itching) associated with the skin lesions. Unlike psoriasis, Bazex Syndrome lesions are particularly notable for their distribution and association with an underlying malignancy.

Etiology

The exact cause of Bazex Syndrome is unknown, but it is believed to be an immunologic response to a neoplasm, most commonly squamous cell carcinoma of the upper aerodigestive tract. Other malignancies have been reported in association with Bazex Syndrome, including adenocarcinomas and lymphomas, but these are less common.

Diagnosis

Diagnosis of Bazex Syndrome is primarily clinical, based on the characteristic skin findings and the presence of an associated malignancy. Skin biopsy can help confirm the diagnosis by showing features similar to psoriasis, including parakeratosis and acanthosis. However, the definitive diagnosis often requires the identification of an underlying tumor, which may involve a variety of imaging studies and possibly endoscopic examinations.

Treatment

The primary treatment for Bazex Syndrome involves addressing the underlying malignancy. Improvement and even resolution of the skin lesions have been reported following successful treatment of the associated cancer. Symptomatic treatment for the skin lesions may include topical steroids and emollients. In some cases, systemic treatments such as retinoids have been used.

Prognosis

The prognosis of Bazex Syndrome is closely tied to the underlying malignancy. Early detection and treatment of the associated cancer can lead to significant improvement in the skin manifestations and overall prognosis.

Epidemiology

Bazex Syndrome is extremely rare, with a limited number of cases reported in the medical literature. It predominantly affects middle-aged to elderly males.