Dermatology

Acrokeratosis Paraneoplastica (Bazex’s Syndrome)

[b1]Acrokeratosis Paraneoplastica (Bazex Syndrome) ICD-9 701.1

Are You Confident of the Diagnosis?

Characteristic findings on physical examination

A typical patient will be a man over 40 years old who has red psoriasiform/scaly plaques distributed on the ears (79%), nails (75%), nose (63%), fingers (61%), hands (57%), and feet (50%). (Figure 1). A history of smoking or heavy alcohol use, which predispose to esophageal cancer (Figure 2) , is supportive of this paraneoplastic eruption.

Figure 1.

Basex syndrome of the helix.

Figure 2.

Esophageal SCC associated with Bazex (for interest as this is SCC of esophagus)

Expected results of diagnostic studies

Common findings on histopathology include acanthosis, psoriasiform epidermal hyperplasia, hyperkeratosis, parakeratosis, and perivascular lymphocytic infiltrate. Less common but helpful are dyskeratotic keratinocyts and vacuolar degeneration of the basal epidermal layer (Figure 3).

Figure 3.

Histology of Basex.

With respect to imaging studies, A full examination of the upper aerodigestive tract is most helpful. This includes a chest x-ray followed by studies as , to include computed tomography (CT) scan and endoscopy (Figure 4). Less commonly, other cancers are associated; therefore, additional screening for colon cancer, lymphoma, bladder, prostate, uterine, vulvar, and neuroendocrine tumors may be necessary.

Figure 4.

CT scan showing radiologic findings in Bazex.

Diagnosis confirmation

The differential diagnosis includes:

Psoriasis- especialy hand and foot psoriasis. Psoriasis responds to topical corticosteroids. There can be oil spots and pits on the nails instead of simply hyperkeratosis. The ears and nose are not commonly involved in psoriasis. Biopsy of psoriasis does not show necrotic keratinocytes.

Howel Evans syndrome (tylosis). An autosomal dominant genodermatosis that has esophageal cancer and a focal nonepidermolytic palmoplantar keratoderma associated with it. This runs in families and has been located to chromosome 17q25.

Dermatophytosis. KOH and culture will confirm a dermatophyte infection

Allergic contact dermatitis (ACD). History and patch testing can be used to identify ACD.

Who is at Risk for Developing this Disease?

This is a rare paraneoplastic condition occurring mainly in men over 40 years of age. It is generally associated with cancer of the upper aerodigestive tract but can occur with other cancers.

What is the Cause of this Disease?

Etiology

The etiology is unknown. Proposed mechanisms include reaction to secreted epidermal growth factors, tumor specific antibodies that react to the skin basement membrane, or zinc deficiency.

Pathophysiology

Pathophysiology consists of keratinocyte proliferation with resultant parakeratosis and acanthosis.

Systemic Implications and Complications

The recognition of acrokeratosis paraneoplastica (Bazex’s syndrome) should lead to an appropriate work-up for malignancy. Certainly, those of the upper aerodigestive system are most common, but others have been reported.

Treatment Options

Treatment of the cancer generally is effective at treating acrokeratosis paraneoplastica. Bolognia showed that 93% of patients improved significantly (or resolved) when the underlying neoplasm was treated. Recurrence of skin lesions can signal recurrence of the tumor. Topical therapies are generally ineffective, a fact which can be helpful in suggesting the diagnosis.

Optimal Therapeutic Approach for this Disease

Treatment of the underlying cancer is the best approach for treatment of acrokeratosis paraneoplastica. Supportive therapies such as emollients and antihistamines can be used to help with symptomatic relief. Phototherapy may also be beneficial.

Patient Management

Recurrence of acrokeratosis paraneoplastica would be a concern for recurrence of the associated cancer. We follow our patients with a yearly skin exam to assess for any evidence of disease. Prior to removal of the cancer, symptoms can be treated with skin-directed therapy used for psoriasis. Zinc supplementation may be warranted.

Unusual Clinical Scenarios to Consider in Patient Management

Remember that in rare cases, acrokeratosis paraneoplastica can have significant overlap with common skin disease, mainly psoriasis. When treating chronic plaque psoriasis of the palms and soles which is not responding to topical therapies, consider acrokeratosis paraneoplastica. Moreover scaly plaques on the ears or nose in association with classic psoriatic lesions on the hands and feet should raise concern for acrokeratosis paraneoplastica.

What is the Evidence?

Poligone, B, Christensen, SR, Lazova, R, Heald, PW. "Bazex syndrome (acrokeratosis paraneoplastica)". Lancet. vol. 369. 2007 Feb 10. pp. 530.

(Case report of patient with classical features and a review of the literature.)

Stone, SP, Buescher, LS. "Life-threatening paraneoplastic cutaneous syndromes". Clin Dermatol. vol. 23. 2005 May-Jun. pp. 301-6.

(Review of paraneoplastic skin diseases.)

Bolognia, JL. "Bazex syndrome: acrokeratosis paraneoplastica". Semin Dermatol. vol. 14. 1995 Jun. pp. 84-9.

(Largest review of the literature with 113 cases reviewed. Statistical analysis with largest data set.)

Bolognia, JL, Brewer, YP, Cooper, DL. "Bazex syndrome (acrokeratosis paraneoplastica). An analytic review". Medicine (Baltimore). vol. 70. 1991 Jul. pp. 269-80.

(Second largest review with 93 patients.)

Bazex, A, Griffiths, A. "Acrokeratosis paraneoplastica--a new cutaneous marker of malignancy". Br J Dermatol. vol. 103. 1980 Sep. pp. 301-6.

(Initial description of acrokeratosis paraneoplastica (Bazex Syndrome) by Bazex in the English language.)

Lucker, GPH, Steijlen, PM. "Acrokeratosis paraneoplastica (Bazex syndrome) occurring with acquired ichthyosis in Hodgkin’s disease". Br J Dermatol. vol. 133. 1995. pp. 322-5.

(Case report of two patients with Hodgkin’s Disease associated Bazex Syndrome. Authors hypothesize transforming growth factor-a may play a role in pathogenesis.)

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. vol. 11. 2007 Mar-Apr. pp. 78-83.

(Case report with associated zinc deficiency.)
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