Acrokeratosis Paraneoplastica

Updated: Jan 23, 2017
  • Author: Katherine R Garrity, MD; Chief Editor: William D James, MD  more...
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Overview

Background

Acrokeratosis paraneoplastica is a rare acral psoriasiform dermatosis associated with internal malignancy, most frequently squamous cell carcinoma (SCC) of the upper aerodigestive tract. The syndrome of acrokeratosis paraneoplastica typically precedes the diagnosis of malignancy. Acrokeratosis paraneoplastica evolves through 3 stages, which are discussed more under in History. Note the images below.

Acrokeratosis paraneoplastica. A 67-year-old woman Acrokeratosis paraneoplastica. A 67-year-old woman presented with scaly plaques of the hands, feet, ears, and nose associated with esophageal squamous cell carcinoma. The eruption resolved with resection of the cancer. Image courtesy of Ronald Grimwood, MD.
Acrokeratosis paraneoplastica. A 67-year-old woman Acrokeratosis paraneoplastica. A 67-year-old woman presented with scaly plaques of the hands, feet, ears, and nose associated with esophageal squamous cell carcinoma. The eruption resolved with resection of the cancer. Image courtesy of Ronald Grimwood, MD.

The term Bazex syndrome describes 2 different entities, both described by Bazex and colleagues: (1) acrokeratosis paraneoplastica and (2) the genetic syndrome of basal cell carcinomas, follicular atrophoderma, hypotrichosis, and disorders of sweating. Note the images below.

Bazex syndrome. Acquired palmar keratoderma in a w Bazex syndrome. Acquired palmar keratoderma in a woman with a history of breast cancer and recent primary lung cancer. Courtesy of Jeffrey J. Meffert, MD.
Bazex syndrome. Violaceous psoriasiform dermatitis Bazex syndrome. Violaceous psoriasiform dermatitis on the ankles. Lung cancer appeared to be in remission; both keratoderma and psoriasiform plaques resolved quickly with clobetasol ointment. Courtesy of Jeffrey J. Meffert, MD.
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Pathophysiology

The pathophysiology of acrokeratosis paraneoplastica is not understood. Proposed mechanisms include cross-reactivity between skin and tumor antigens, the action of tumor-produced growth factors, and even zinc deficiency. [1, 2]

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Epidemiology

Frequency

Approximately 140 cases of acrokeratosis paraneoplastica have been reported in the literature. [3]

Sex

In one review, only 12 of 140 cases of acrokeratosis paraneoplastica were in women. [3]

Age

In one review, the mean age of onset for acrokeratosis paraneoplastica was 61 years. [4]

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Prognosis

In most reported cases of acrokeratosis paraneoplastica, cervical lymph node metastases are present at the time of diagnosis, usually indicating a poor prognosis. [4]  Morbidity and mortality in acrokeratosis paraneoplastica are related directly to the underlying neoplasm. The skin lesions of acrokeratosis paraneoplastica rarely improve without successful treatment of the underlying malignancy. [4]

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