Bazex

Bazex, also known as paraneoplastic acrokeratosis, is a rare genodermatosis. It is characterized by a triad of skin manifestations: hyperkeratosis and scaling of the palms and soles, and follicular hyperkeratosis and scaling on the extremities. Nail changes, such as onychodystrophy, are also common. Bazex syndrome is typically inherited in an autosomal dominant pattern.

The genetic basis of Bazex syndrome is primarily linked to mutations in the BCL11B gene, which plays a role in hair follicle development and epidermal differentiation. In some cases, mutations in other genes may also be implicated. The onset of symptoms usually occurs in infancy or early childhood. The characteristic skin lesions can be persistent and may cause discomfort, particularly in areas subject to friction.

Diagnosis is usually made based on clinical presentation and a family history of similar symptoms. Genetic testing can confirm the diagnosis. There is no cure for Bazex syndrome, and management focuses on symptomatic relief. Topical treatments, such as emollients and keratolytics, can help manage the hyperkeratosis and scaling. In more severe cases, systemic medications may be considered. Regular dermatological follow-up is recommended to monitor the condition and manage any complications.