Case Report
Acrokeratosis paraneoplastica (Bazex's syndrome): Association with liposarcoma

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Acrokeratosis paraneoplastica (Bazex's syndrome) is a rare obligate paraneoplastic dermatosis characterized by erythematosquamous lesions localized symmetrically at the acral sites. The condition almost exclusively affects Caucasian men older than 40 years. It is usually associated with primary malignant neoplasms of the upper aerodigestive tract. In most cases, the skin changes precede the clinical manifestation of the underlying neoplasm. The dermatosis can be cured only by removal of the underlying carcinoma. We describe a case of acrokeratosis paraneoplastica associated with a retroperitoneal liposarcoma in a 71-year-old Caucasian man. The liposarcoma was surgically removed but recurred several times, with acrokeratosis paraneoplastica showing a parallel development. We, therefore, add liposarcoma to the growing list of malignant neoplasms associated with acrokeratosis paraneoplastica.

Section snippets

Case report

A 71-year-old man, in good general health, was referred to our department with a diagnosis of suggested psoriasis vulgaris. He underwent extirpation of a liposarcoma in the right retroperitoneum 4 months before admission. Six weeks after operation, the patient developed a psoriasiform and moderately itching dermatitis.

He showed, typically for acrokeratosis paraneoplastica, erythematosquamous papules and plaques distributed symmetrically in the region of the knuckles, fingernails, fingertips,

Discussion

Bazex et al1, 6 described acrokeratosis paraneoplastica as evolving in 3 stages. Stage I is marked by symmetrically squamous, psoriasiform erythema of the tips of fingers and toes (nail changes frequently occurring), followed by scaling of the back of the nose and the helical region. Symptoms of the malignant disease are absent here. Stage II consists of skin lesions spread in a centripetal pattern. At this time, early general tumor-related symptoms are observed. Stage III is marked by the

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    Citation Excerpt :

    Palmoplantar hyperkeratosis is more common at the pressure points of the palm and does not affect the central area. The lesions can precede the detection of the underlying malignancy by a year; commonly associated malignancies are squamous cell carcinomas of the larynx, esophagus, and lung,48,49 but some cases have been reported in other malignancies, including ductal breast adenocarcinoma, cholangiocarcinoma, adenocarcinoma of the colon and prostate,50 lymph node metastasis in the neck, and liposarcoma.51 Several factors have been implicated in the pathogenesis of acrokeratosis paraneoplastica of Bazex, including antigen cross-reactivity between the tumor and the skin, production of a keratinocyte growth factor, vitamin A deficiency, and genetic susceptibility.

  • Miscellaneous conditions

    2009, Weedon's Skin Pathology: Third Edition
  • Bazex syndrome or dermatomyositis?

    2007, Journal of the American Academy of Dermatology
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Funding sources: None.

Conflicts of interest: None identified.

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