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Acrokeratosis Paraneoplastica Clinical Presentation

  • Author: Katherine R Garrity, MD; Chief Editor: William D James, MD  more...
 
Updated: Jan 06, 2015
 

History

A diagnostic algorithm for acrokeratosis paraneoplastica has been proposed by Valdivielso, et al. At first suspicion of acrokeratosis paraneoplastica, a detailed history should be taken.[5]

The physician should inquire regarding risk factors for malignancy, including smoking habit, alcohol consumption, and family history. Upon further questioning, patients with suspected acrokeratosis paraneoplastica may admit to mild constitutional symptoms, weight loss, and other nonspecific findings of internal malignancy.

In one review of acrokeratosis paraneoplastica, when skin findings preceded the diagnosis of malignancy, they were present for an average of 1 year.[4] The interval between acrokeratosis paraneoplastica skin manifestations and tumor diagnosis has been reported to be as long as 3 years in some cases.[6] Signs of acrokeratosis paraneoplastica precede the detection of the primary malignancy in 65-70% of instances. Of acrokeratosis paraneoplastica patients, 10-15% have lesion appearance after the diagnosis of malignancy. In the remaining 15-25% of acrokeratosis paraneoplastica cases, both occur simultaneously.[7]

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Physical

A complete physical examination should be performed in all patients suspected of having acrokeratosis paraneoplastica. This should include a thorough head and neck examination, including endoscopic examination, and pelvic examination in women.[4, 5]

Cutaneous manifestations of acrokeratosis paraneoplastica include the following:

  • Symmetrical, acral, scaly, red-to-violaceous plaques or patches
  • Acrally distributed, hyperpigmented patches possible in persons with darker skin types
  • Involvement of the fingers, distal hands, feet, nose, and helices of the ears: Isolated involvement of the helices is particularly suggestive of acrokeratosis paraneoplastica. [8]
  • Involvement of the cheeks, trunk, elbows, knees, palms, and soles in advanced disease
  • Nail dystrophy, horizontal and vertical ridging (75%), subungual hyperkeratosis, nail plate atrophy, tender periungual erythema, and cuticle loss
  • Swelling of the digits with a blue-to-violet discoloration
  • Bullae of the hands and feet

There have been case reports of more unusual presentations, including oral ulcerations[9] and generalized erythroderma.[10]

Three stages of lesion evolution have been described for acrokeratosis paraneoplastica. After performing the physical examination, the patient can be staged as follows[11] :

  • Stage 1: Characterized by poorly defined psoriasiform plaques involving the ears, nose, nails, fingers, and toes; nail folds become dystrophic at this stage
  • Stage 2: Characterized by involvement of larger and more proximal regions; violaceous keratoderma with central clearing is seen over the cheeks, palms, and/or soles
  • Stage 3: Characterized by involvement of the legs, knees, thighs, and arms

Attention should also be paid to signs of other paraneoplastic syndromes, because patients may present with more than one.[12] Other physical examination findings suggesting the possibility of neoplasia are as follows:

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Causes

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, and skin (including SCC in situ)[13] . 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,[3] liposarcoma,[14] well-differentiated thymic carcinoma,[15] and adenocarcinoma of multiple primary sites including tonsils.[16]

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Contributor Information and Disclosures
Author

Katherine R Garrity, MD Resident Physician, Department of Dermatology, University of Wisconsin School of Medicine and Public Health

Katherine R Garrity, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

Coauthor(s)

Daniel D Bennett, MD Assistant Professor, Department of Dermatology, University of Wisconsin School of Medicine and Public Health

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

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

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

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD Associate 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, Texas Dermatological Society

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

Karen D Allen, MD Consulting Dermatologist, Private Practice

Karen D Allen, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Cary Chisholm, MD Dermatopathology Fellow, Department of Dermatology, University of Texas Southwestern Medical Center

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.

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. 2007 Mar-Apr. 11(2):78-83. [Medline].

  2. Rodrigues IA Jr, Gresta LT, Cruz RC, Carvalho GG, Moreira MH. Bazex syndrome. An Bras Dermatol. 2013 Nov-Dec. 88(6 Suppl 1):209-11. [Medline]. [Full Text].

  3. 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. 2006 Dec. 33(12):850-4. [Medline].

  4. 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. 2007 Mar-Apr. 11(2):78-83. [Medline].

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

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

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

  8. Lorette G, Samimi M. [Hands cutaneous exam]. Presse Med. 2013 Dec. 42(12):1582-95. [Medline].

  9. Santos-Silva AR, Correa MB, Vargas PA, Almeida OP, Lopes MA. Bazex syndrome (acrokeratosis paraneoplastica) diagnosed in a patient with oral persistent ulcerations. Head Neck Pathol. 2010 Dec. 4(4):312-7. [Medline]. [Full Text].

  10. Protopsaltis I, Drossou A, Katsantonis I, Roussos N, Manoludaki K, Arvanitis M, et al. Breast cancer presenting as paraneoplastic erythroderma: an extremely rare case. Case Rep Med. 2014. 2014:351065. [Medline]. [Full Text].

  11. Chapireau D, Adlam D, Cameron M, Thompson M. Paraneoplastic syndromes in patients with primary oral cancers: a systematic review. Br J Oral Maxillofac Surg. 2010 Jul. 48(5):338-44. [Medline].

  12. da Rosa AC, Pinto GM, Bortoluzzi JS, Duquia RP, de Almeida HL Jr. Three simultaneous paraneoplastic manifestations (ichthyosis acquisita, Bazex syndrome, and Leser-Trélat sign) with prostate adenocarcinoma. J Am Acad Dermatol. 2009 Sep. 61(3):538-40. [Medline].

  13. Lau SY, Khow KS, Yong TY. Medical image. Acrokeratosis paraneoplastica with in-situ squamous cell carcinoma. N Z Med J. 2014 Feb 14. 127(1389):86-7. [Medline].

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

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

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

  17. 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. 2006 Apr. 97(3):196-9. [Medline].

  18. 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. 2009 Mar. 47(2):138-9. [Medline].

  19. Medenica L, Gajic-Veljic M, Skiljevic D, Pesko P. Acrokeratosis paraneoplastica Bazex syndrome associated with esophageal squamocellular carcinoma. Vojnosanit Pregl. 2008 Jun. 65(6):485-7. [Medline].

 
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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 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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