Acrokeratosis paraneoplastica

Acrokeratosis paraneoplastica (also known as Bazex syndrome) is a rare paraneoplastic syndrome associated with squamous cell carcinomas, typically of the upper aerodigestive tract. It is characterized by distinctive skin lesions that primarily affect the hands and feet. This condition is considered a paraneoplastic syndrome, which means it is a disorder triggered by an immune system response to a neoplasm (tumor) in the body.

Symptoms and Signs

The hallmark of Acrokeratosis paraneoplastica is the development of psoriasiform plaques, primarily on the acral parts of the body - the hands and feet. These lesions can also extend to the elbows, knees, and ears. Patients may also experience nail changes, including thickening and discoloration. The skin changes are often symmetrical and can be accompanied by erythema (redness of the skin) and scaling.

Etiology

Acrokeratosis paraneoplastica is most commonly associated with squamous cell carcinoma of the upper aerodigestive tract, including the larynx, pharynx, and esophagus. The exact mechanism by which the tumor induces the skin manifestations is not fully understood, but it is believed to involve an immune-mediated response.

Diagnosis

The diagnosis of Acrokeratosis paraneoplastica is primarily clinical, based on the characteristic skin findings and the presence of an associated malignancy. Skin biopsy can help confirm the diagnosis by revealing psoriasiform epidermal hyperplasia and other characteristic histopathological features. It is also essential to search for an underlying malignancy in patients diagnosed with this syndrome, which may involve imaging studies and endoscopic examinations.

Treatment

Treatment of Acrokeratosis paraneoplastica focuses on addressing the underlying malignancy. The resolution of skin lesions is often observed following successful treatment of the associated cancer. Symptomatic treatment may also be provided for the skin lesions, including the use of topical steroids and emollients to reduce inflammation and scaling.

Prognosis

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

See Also

• Paraneoplastic syndromes
• Squamous cell carcinoma
• Psoriasis