Acrokeratosis Paraneoplastica

Updated: Nov 05, 2021
  • Author: Katherine R Garrity, MD; Chief Editor: William D James, MD  more...
  • Print
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. [1] 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.
Next:

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. [2, 3]

Previous
Next:

Etiology

Most acrokeratosis paraneoplastica cases are associated with squamous cell carcinoma (SCC) of the upper one third of the respiratory or GI tracts (ie, oropharynx/larynx, lungs, esophagus).

Case reports also describe SCC of the thymus, vulva, cervix, [4] and skin [5] (including SCC in situ). [6] Reports have also described acrokeratosis paraneoplastica associated with SCC with an unknown primary tumor.

Other reported associated tumors include poorly differentiated carcinoma (not otherwise specified), small cell carcinoma of the lung, lymphoma, ductal carcinoma of the breast, carcinoid, multiple myeloma, transitional cell carcinoma of the bladder, cholangiocarcinoma, [7] liposarcoma, [8] well-differentiated thymic carcinoma, [9] and adenocarcinoma of multiple primary sites including tonsils and pancreas. [10, 11]

Previous
Next:

Epidemiology

Frequency

Approximately 140 cases of acrokeratosis paraneoplastica have been reported in the literature. [7]

Sex

In one review, only 12 of 140 cases of acrokeratosis paraneoplastica were in women. [7]

Age

In one review, the mean age of onset for acrokeratosis paraneoplastica was 61 years. [12]

Previous
Next:

Prognosis

In most reported cases of acrokeratosis paraneoplastica, cervical lymph node metastases are present at the time of diagnosis, usually indicating a poor prognosis. [12]  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. [12]

Previous