Acrokeratosis Neoplastica 

  • Author: Cary Chisholm, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Apr 9, 2010
 

Background

Acrokeratosis neoplastica 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 neoplastica typically precedes the diagnosis of malignancy. Acrokeratosis neoplastica evolves through 3 stages, which are discussed more under in History. Note the images below.

Acrokeratosis neoplastica. A 67-year-old woman preAcrokeratosis neoplastica. 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 neoplastica. A 67-year-old woman preAcrokeratosis neoplastica. 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: acrokeratosis neoplastica and 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 wBazex 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 dermatitisBazex 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 neoplastica 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]

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Epidemiology

Frequency

United States

Approximately 140 cases of acrokeratosis neoplastica have been reported in the literature.[2]

Mortality/Morbidity

Morbidity and mortality in acrokeratosis neoplastica are related directly to the underlying neoplasm. The skin lesions of acrokeratosis neoplastica rarely improve without successful treatment of the underlying malignancy.[3]

Sex

In one review, only 12 of 140 cases of acrokeratosis neoplastica were in women.[2]

Age

In one review, the mean age of onset for acrokeratosis neoplastica was 61 years.[3]

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Proceed to Clinical Presentation
 
 
Contributor Information and Disclosures
Author

Cary Chisholm, MD  Resident Physician, Department of Pathology, Texas A&M Health Science Center College of Medicine

Cary Chisholm, MD is a member of the following medical societies: College of American Pathologists, Texas Medical Association, and United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Coauthor(s)

Daniel D Bennett  MD, Assistant Professor

Daniel D Bennett is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Dermatology Foundation, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Jean-Hilaire Saurat, MD  Chair, Professor, Department of Dermatology, University of Geneva, Switzerland

Jean-Hilaire Saurat, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD  Assistant Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Karen Allen, MD, to the development and writing of this article. The authors and editors of eMedicine would also like to gratefully acknowledge the contributions of previous Chief Editor, William James, MD, to the development and writing of this article.

References

  1. Taher M, Grewal P, Gunn B, Tonkin K, Lauzon G. Acrokeratosis paraneoplastica (Bazex syndrome) presenting in a patient with metastatic breast carcinoma: possible etiologic role of zinc. J Cutan Med Surg. Mar-Apr 2007;11(2):78-83. [Medline].

  2. Karabulut AA, Sahin S, Sahin M, Eksioglu M, Ustün H. Paraneoplastic acrokeratosis of Bazex (Bazex's syndrome): report of a female case associated with cholangiocarcinoma and review of the published work. J Dermatol. Dec 2006;33(12):850-4. [Medline].

  3. Taher M, Grewal P, Gunn B, Tonkin K, Lauzon G. Acrokeratosis paraneoplastica (Bazex syndrome) presenting in a patient with metastatic breast carcinoma: possible etiologic role of zinc. J Cutan Med Surg. Mar-Apr 2007;11(2):78-83. [Medline].

  4. Valdivielso M, Longo I, Suarez R, Huerta M, Lazaro P. Acrokeratosis paraneoplastica: Bazex syndrome. J Eur Acad Dermatol Venereol. May 2005;19(3):340-4. [Medline].

  5. Crucitti A, Feliciani C, Grossi U, La Greca A, Porziella V, Giustacchini P. Paraneoplastic acrokeratosis (bazex syndrome) in lung cancer. J Clin Oncol. Dec 20 2009;27(36):e266-8. [Medline].

  6. Ljubenovic MS, Ljubenovic DB, Binic II, Jankovic AS, Jovanovic DL. Acrokeratosis paraneoplastica (Bazex syndrome). Indian J Dermatol Venereol Leprol. May-Jun 2009;75(3):329. [Medline].

  7. Chapireau D, Adlam D, Cameron M, et al. Paraneoplastic syndromes in patients with primary oral cancers: A systematic review. Br J Oral Maxillofacial Surg. 2009;epub ahead of print.

  8. da Rosa ACM, Pinto GM, Bortoluzzi JS, et al. Three simultaneous paraneoplastic manifestations (ichthyosis acquisita, Bazex syndrome, and Leser-Trelat sign) with prostate adenocarcinoma. J Am Acad Dermatol. 2009;61:538-540.

  9. Sator PG, Breier F, Gschnait F. Acrokeratosis paraneoplastica (Bazex's syndrome): association with liposarcoma. J Am Acad Dermatol. Dec 2006;55(6):1103-5. [Medline].

  10. Chave TA, Bamford WM, Harman KE. Acrokeratosis paraneoplastica associated with recurrent metastatic thymic carcinoma. Clin Exp Dermatol. Jul 2004;29(4):430-2. [Medline].

  11. Ali M, Keir M, Dodd H, Cerio R. Flexural Bazex syndrome associated with tonsillar adenocarcinoma. J Drugs Dermatol. Sep-Oct 2004;3(5):557-9. [Medline].

  12. Cabanillas M, Perez-Perez L, Sanchez-Aguilar D, Fernandez-Redondo V, Toribio J. [Acrokeratosis paraneoplastica with bullous lesions associated with esophageal squamous cell carcinoma]. Actas Dermosifiliogr. Apr 2006;97(3):196-9. [Medline].

  13. Fasanmade A, Farrell K, Perkins CS. Bazex syndrome (acrokeratosis paraneoplastica): persistence of cutaneous lesions after successful treatment of an associated oropharyngeal neoplasm. Br J Oral Maxillofac Surg. Mar 2009;47(2):138-9. [Medline].

  14. Medenica L, Gajic-Veljic M, Škiljevic D, et al. Acrokeratosis paraneoplastica Bazex syndrome associated with eophageal squamocellular carcinoma. Vojnosanit Pregl. 2008;65:485-487.

 
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Acrokeratosis neoplastica. 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 neoplastica. 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.
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 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.
At this power, a patchy lichenoid infiltrate of predominantly lymphocytes can be seen underneath an epidermis with psoriasiform hyperplasia and serum crust in the parakeratotic cornified layer (hematoxylin and eosin, 100X).
Focal vacuolar interface change is seen with associated pigment incontinence and exocytosis of lymphocytes (hematoxylin and eosin, 200X).
 
 
 
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