AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Background

Acrokeratosis neoplastica (AN) is a rare psoriasiform dermatosis that is associated consistently with internal malignancy, usually squamous cell carcinoma (SCC) of the upper aerodigestive tract. Skin manifestations include acute onset of onychodystrophy and violaceous scaly patches on the helices, nose, and malar and acral surfaces. Usually, skin manifestations precede diagnosis of the malignancy.

The term Bazex syndrome describes 2 different entities: AN and the genetic syndrome of basal cell carcinomas, follicular atrophoderma, hypotrichosis, and hypohidrosis or hyperhidrosis.

Pathophysiology

The skin manifestations of AN parallel the disease course, suggesting the presence of circulating antibodies to tumor antigens. Cytokines may play a role.

Frequency

United States

Approximately 125 cases have been reported in the literature.

Mortality/Morbidity

• Mortality and morbidity are related directly to the available treatment options for the underlying neoplasm.
• In most reported cases, cervical lymph node metastases are present at the time of diagnosis, usually indicating a poor prognosis.

Sex

In one review, 105 of 113 cases were in males.

Age

Mean age of onset is 61 years.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

History

• Skin findings usually begin with scaling dermatosis of the fingers and toes that quickly spreads to the nails (Media File 1).
• • Nails can show severe dystrophy with painful paronychia, subungual debris, and linear striations. In the absence of evidence of fungal infection, acute onset of this dystrophy indicates AN.
  • Involvement of the ear helices with a violaceous scaly eruption is a constant finding.
  • With further progression, involvement of the cheeks and nose occurs.
  • In the second stage of AN, a palmoplantar keratoderma is present that also can be violaceous in color.
  • The final stage may involve similar skin findings extending to the trunk, extremities, and scalp (Media File 2).
  • Although the paronychia of the nails may be painful and tender and cracking and fissuring of the fingertips may be seen, the papulosquamous lesions typically are asymptomatic.
• On further questioning, patients may admit to mild constitutional symptoms, weight loss, and other nonspecific findings of internal malignancy.

Causes

• Most AN cases are associated with SCC of the upper one third of the respiratory tract or the GI tract (oral cavity, pharynx, larynx, esophagus).
• Generally, the lymph nodes already are involved at diagnosis.
• Case reports also exist of SCC of the thymus and vulva and of poorly differentiated SCC of the cervical lymph nodes with an unknown primary tumor.
• Rare associated cancers reported include anaplastic small cell carcinoma of the lung and adenocarcinoma of the uterus.

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Other Problems to be Considered

Drug eruption
Photodermatitis

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Lab Studies

• Physical examination may indicate the need for specific laboratory studies in the search for underlying malignancy. Other screening laboratory studies may reveal evidence of GI or renal malignancies.
• • General chemistry panel to evaluate for occult hepatic or renal disease
  • Urinalysis
  • Complete blood cell count
  • Test for occult blood in the stool
• KOH (negative in AN) to exclude fungal infection, which is in the differential of AN.

Imaging Studies

• Chest radiography
• CT scanning or MRI of specific body sites or as part of a general radiologic screening
• Pelvic ultrasonography
• Mammography

Other Tests

• Lymph node biopsy if adenopathy is present
• Bronchoscopy
• Upper GI endoscopy
• Rhinolaryngoscopy

Histologic Findings

Skin biopsy specimens often reveal nonspecific changes of a psoriasiform or spongiotic dermatitis. Some authors have noted vacuolar degeneration of the basal cell layer with occasional bullae formation.

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Medical Care

• Treatment depends on the type and stage of the underlying neoplasm. Often, treating the neoplasm improves the cutaneous disease. If the neoplasm is cured, skin signs abate.
• While specific treatment of the skin improves the eruption, this usually is not curative.
• • Topical corticosteroids may improve scaly lesions.
  • Therapy with cholecalciferol also has been reported to be of benefit.

Consultations

• Consultation with an internal medicine specialist for malignancy workup is appropriate.
• Consultation with a hematologist, oncologist, otolaryngologist and/or gastroenterologist also can assist with focused evaluations.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

The goal of pharmacotherapy is to improve scaly lesions, reduce morbidity, and prevent complications.

Drug Category: Corticosteroids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.

Drug Category: Dietary Supplements

For treatment of vitamin D deficiency or prophylaxis of vitamin D deficiency. Vitamin D and its analogs have profound effects on cellular proliferation and differentiation in addition to immunomodulatory effects. Calcipotriene, a vitamin D analog, is a well-accepted treatment for psoriasis, which is a condition that shares some similarities with AN. However, the reports of efficacy in the treatment of AN with vitamin D are anecdotal.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Prognosis

• In most reported cases of AN, cervical lymph node metastases are present at the time of diagnosis, usually indicating a poor prognosis.

MULTIMEDIA

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

Media file 1:  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.
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Media type:  Photo

Media file 2:  Bazex syndrome. Violaceous psoriasiform dermatitis on the ankles (same patient as in Image 1). Lung cancer appeared to be in remission; both keratoderma and psoriasiform plaques resolved quickly with clobetasol ointment. Courtesy of Jeffrey J Meffert, MD.
	View Full Size Image
Media type:  Photo

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Multimedia
• References

• Bolognia JL. Bazex syndrome: acrokeratosis paraneoplastica. Semin Dermatol. Jun 1995;14(2):84-9. [Medline].
• Boudoulas O, Camisa C. Paraneoplastic acrokeratosis: Bazex syndrome. Cutis. Jun 1986;37(6):449-53. [Medline].
• Richard M, Giroux JM. Acrokeratosis paraneoplastica (Bazex'' syndrome). J Am Acad Dermatol. Jan 1987;16(1 Pt 2):178-83. [Medline].

Article Last Updated: Aug 17, 2005