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Acrokeratosis Neoplastica
Updated: Jul 28, 2008
Introduction
Background
Acrokeratosis neoplastica (AN) is a rare acral psoriasiform dermatosis associated with internal malignancy, most frequently squamous cell carcinoma (SCC) of the upper aerodigestive tract. The syndrome typically precedes the diagnosis of malignancy.
The term Bazex syndrome describes 2 different entities, both described by Bazex and colleagues: AN and the genetic syndrome of basal cell carcinomas, follicular atrophoderma, hypotrichosis, and disorders of sweating.
The Medscape Head and Neck Cancer Resource Center and the eMedicine article Squamous Cell Carcinoma may be of interest.
Pathophysiology
The pathophysiology of AN 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
Frequency
United States
Approximately 140 cases have been reported in the literature.2
Mortality/Morbidity
Mortality and morbidity are related directly to the underlying neoplasm. The skin lesions of AN rarely improve without successful treatment of the underlying malignancy.3
Sex
In one review, only 12 of 140 cases were in women.2
Age
In one review, the mean age of onset was 61 years.3
Clinical
History
- Patients present with asymptomatic, acrally located psoriasiform or hyperpigmented lesions. The lesions are treatment resistant.
- As the tumor progresses, the lesions of AN may spread and may involve the cheeks, elbows, knees, and trunk.
- In one review, when skin findings preceded the diagnosis of malignancy, they were present for an average of 1 year.3
- The physician should inquire regarding risk factors for malignancy, including smoking habit, alcohol consumption, and family history. Upon further questioning, patients may admit to mild constitutional symptoms, weight loss, and other nonspecific findings of internal malignancy.
Physical
- Cutaneous manifestations of AN are as follows:
- Symmetrical, acral, scaly, red-to-violaceous plaques or patches
- Involvement of the fingers, distal hands, feet, nose, and helices of the ears: Isolated involvement of the helices is particularly suggestive of AN.
- Involvement of the cheeks, trunk, elbows, knees, palms, and soles in advanced disease
- Nail dystrophy
- Swelling of the digits with a blue-to-violet discoloration
- Acrally distributed, hyperpigmented patches possible in persons with darker skin types
- Bullae of the hands and feet
- Other physical examination findings suggesting paraneoplasia are as follows:
- Ichthyosis
- Pruritus
- Sign of Leser-Trélat
- Clubbing
- Dermatomyositis
- A complete physical examination should be performed in all patients. This should include a thorough head and neck examination, including endoscopic examination, and pelvic examination in women.3
Causes
- Most AN cases are associated with 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 and of SCC with an unknown primary tumor.
- Other reported associated tumors include poorly differentiated carcinoma, adenocarcinoma of multiple primary sites, small cell carcinoma of the lung, lymphoma, ductal carcinoma of the breast, carcinoid, multiple myeloma, transitional cell carcinoma of the bladder, well-differentiated thymic carcinoma, and cholangiocarcinoma.2
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| Treatment & Medication: Acrokeratosis Neoplastica |
| Follow-up: Acrokeratosis Neoplastica |
| Multimedia: Acrokeratosis Neoplastica |
| References |
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References
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].
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].
Bolognia JL. Bazex syndrome: acrokeratosis paraneoplastica. Semin Dermatol. Jun 1995;14(2):84-9. [Medline].
Valdivielso M, Longo I, Suárez R, Huerta M, Lázaro P. Acrokeratosis paraneoplastica: Bazex syndrome. J Eur Acad Dermatol Venereol. May 2005;19(3):340-4. [Medline].
Further Reading
Keywords
acrokeratosis neoplastica, keratosis, Bazex syndrome, paraneoplastic acrokeratosis
