A series of typical and atypical cases of Bazex syndrome: Identifying the red herring to avoid delaying cancer treatment

Abstract

Acrokeratosis paraneoplastica or Bazex syndrome is a paraneoplastic dermatosis that often presents prior to a diagnosis of an internal malignancy. The symptoms can be significant and include dry and thickened skin on the hands, face, and lower extremities as well as brittle, thickened nails on the hands and feet. These symptoms usually result in presentation to the clinic several months before the manifestation of signs or symptoms of malignancy, and they are also typically resistant to dermatologic management. 1 By the time of cancer diagnosis, most patients have endured months of ineffective dermatologic care as their malignancy was left untreated. The earliest case report of hyperkeratosis of hands and feet with internal malignancy was published in 1922 by Gougerot and Rupp. 2 Acrokeratosis Paraneoplastica or Bazex syndrome, dermatosis associated with internal malignancy, was not recognized until 1965, by Andre Bazex. 3 However, its recognition outside of

Acrokeratosis Paraneoplastica (Bazex Syndrome): A Case Report

“…By now, there are about 150 published cases of Bazex syndrome. The typical patients' characteristics are middle-aged Caucasian men, women are less likely affected [ 8 ]. A systematic review published in 2017 illustrates that in most cases of acrokeratosis paraneoplastica, the underlying neoplasm was squamous cell carcinoma of the upper aerodigestive tract (head-neck cancer, lung) or cervical lymph node metastases with an unknown primary tumor, followed by gastrointestinal and genitourinary tumors.…”

Bazex Syndrome (Acrokeratosis Paraneoplastica): A Narrative Review of Pathogenesis, Clinical Manifestations, and Therapeutic Approaches

“…When these antigens infiltrate the immune system, the body mounts a defensive antibody response. The ensuing antibodies, crafted to neutralize these tumor antigens, might inadvertently cross-react with antigens present on keratinocytes or the basement membrane [ 8 , 9 ]. Alternatively, the body might mount a T-cell-mediated immune response against the tumor antigens, but the cytotoxic T-cells may cross-react with keratinocyte antigens.…”

“…Notably, etretinate, an aromatic retinoid, demonstrated improvement in these skin lesions [ 26 ]. Acitretin has also been effective, providing relief to at least two patients in a documented case series [ 8 ]. Interestingly, one of these patients experienced a recurrence and even progression of cutaneous symptoms and burning sensations after discontinuing acitretin treatment, despite being cancer-free.…”

Bazex Syndrome Associated with Angioimmunoblastic T-cell Lymphoma

“…Inconstant features include variable spongiosis and dyskeratosis. [4][5] While the histologic features are not always diagnostic, the absence of diagnostic features of other conditions and clinicopathologic correlation are key to rule out other entities on the differential diagnosis. In our patient, the presence of confluent parakeratosis excluded the possibility of malignancyassociated ichthyosis.…”