Acrokeratosis paraneoplastica (Bazex Syndrome) - Submitted on: Monday February, 04, 2008

Contributed by:Shaofeng Yan, MD, PhD, N/A

Acrokeratosis paraneoplastica (Bazex Syndrome) is a rare paraneoplastic skin condition

Definition: Acrokeratosis paraneoplastic of Bazex is a rare syndrome which denotes the presence of a skin condition as a marker of an underlying internal malignancy.

Clinical Features: Acrokeratosis paraneoplastica (Bazex Syndrome) is characterized by acral psoriasiform lesions associated with an underlying neoplasm (1, 2). More than half of acrokeratosis paraneoplastica associated malignancies are supradiaphragmatic in origin, such as oropharynx, larynx, esophagus and lung. The skin findings often precede the onset of symptoms of the underlying neoplasm. Diagnosis of this syndrome therefore provides an essential clue for the search of the underlying neoplasm (1). Patients usually present with violaceous or erythematous scaling lesions on the ears, nose, hands and feet, sometimes arms and legs (3). Bullous lesions are less common. Patients may develop violaceous keratoderma on hands and feet. The pathogenesis of this disease is unclear.

Histology: Figures (Click on an image for a larger view) Figure 1: Epidermal hyperplasia with hyperkeratosis and a thick inflammatory scale crust (Hematoxylin and Eosin, 2x). Figure 2: Psoriasiform epidermal hyperplasia with hyperkeratosis, parakeratosis and mild spongiosis. (Hematoxylin and Eosin, 10x). Figure 3. Scattered dyskeratosis within the epidermis (Hematoxylin and Eosin, 40x). Figure 4. Mixed mononuclear cell infiltrate (Hematoxylin and Eosin, 40x). The histological findings of acrokeratosis paraneoplastica are not specific and often show overlap features of psoriasis and chronic spongiotic dermatitis. The commonly described features include acanthosis, psoriasiform epidermal hyperplasia (fig 1 and 2), hyperkeratosis, parakeratosis, dyskeratosis (fig 3), vacuolar interface change of the basal layer and perivascular lymphocytic infiltrate (fig 4).

Treatment: With the fact that Bazex syndrome is an early marker of the underlying subcliniical neoplasm, an early suspicion is of the most importance in order to perform a prompt diagnosis of an underlying malignancy. Thus clinical suspicion of Bazex syndrome should lead to a skin biopsy followed by a complete evaluation of the internal malignancy. The skin lesions usually regress following the treatment of the underlying disease.

Bibliography: 1. Bazex A, Griffiths A. Acrokeratosis paraneoplastica--a new cutaneous marker of malignancy. Br J Dermatol. 1980 Sep;103(3):301-6. Review 2. Jacobsen FK, Abildtrup N, Laursen SO, Brandrup F, Jensen NK. Acrokeratosis paraneoplastica (Bazex' syndrome). Arch Dermatol. 1984 Apr;120(4):502-4. 3. Bolognia JL, Brewer YP, Cooper DL. Bazex syndrome (acrokeratosis paraneoplastica). An analytic review. Medicine (Baltimore). 1991 Jul;70(4):269-80. Review

 

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