Acrokeratosis paraneoplastica (Bazex' syndrome) associated with metastatic squamous cell esophageal carcinoma.

In March 2003, a 55-year-old male with a longstanding history of alcohol abuse and smoking developed diffuse hyperkeratosic cutaneous lesions affecting the nose, toes, and later the fingers and hands (Fig 1). At consultation, the clinical course had been about 4 months. Physical examination revealed ungual involvement (Fig 2) and no other clinical sign. Skin biopsy was noncontributive. Local corticosteroids were initiated, but had no effect. In December 2003, the cutaneous lesions worsened, spreading to the back. The patient lost 7 kg and presented multiple supraclavicular and cervical node enlargements bilaterally. The pathology diagnosis of a nodal biopsy was squamous cell carcinoma. The ear, nose, and throat (ENT) examination was negative. A thoraco-abdomino-pelvic computed tomography revealed thickening of the midthird of the esophagus, presence of enlarged coelio-mesenteric and retroperitoneal nodes, as well as lung and liver metastases. Upper gastrointestinal endoscopy visualized a wide circumferential ulceration of the midthird of the esophagus. The biopsy revealed squamous cell carcinoma, and he was then referred to our department. The diagnosis of squamous cell carcinoma associated with acrokeratosis paraneoplastic (Bazex’ syndrome) was retained. A chemotherapy regimen of cisplatin and flurouracil was delivered. The patient’s general status improved rapidly,withinvolutionofthenodalenlargements,completeregressionof the skin lesions, and restitutio ad integrum of the ungual lesions. The patient nevertheless died in September 2005 after disease progression manifested by a recurrence of the ungual lesions. Bazex’ syndrome is a rare paraneoplastic syndrome affecting the skin described for the first time in 1965. Diagnosis is based on the presence of psoriasiform hyperkeratosic lesions involving the extremities (ears, fingers, toes, nose), but also the trunk and limbs. Functional signs are generally absent (no pruritis). Ungual involvement is common. Bazex’ syndrome has three stages: (1) cutaneous lesions of the ears, fingers, toes, and nails; (2) extension of the skin involvement to the volar aspect of the hand and foot—the neoplastic disease is generally symptomatic at this stage; and (3) in the absence of etiological treatment, the lesions spread to the knees, elbows, and trunk. In 70% of the cases reported in the literature, the cutaneous syndrome precedes the discovery of the neoplasm which generally involves the upper airways or gastrointestinal tract (including ENT) and the lungs. However, case reports have illustrated other neoplastic localizations: bladder, prostate, breast, and liposarcoma. The syndrome progresses in parallel with the neoplasia and subsequent treatment, but does not respond to symptomatic treatments. The formation of the cutaneous lesions might be explained by the production of epidermal growth factor, but solid evidence is lacking. Skin biopsies can be useful to rule out differential diagnoses but generally only disclose nonspecific features (hyperkeratosis, acanthosis, mononuclear cell perivascular infiltration). Clinicians should be aware of this rare syndrome because recognition enables earlier diagnosis of the underlying neoplasia. Patients should undergo a specialized ENT examination and endoscopic explorations with regular surveillance in the event of a negative work-up. Early diagnosis can improve the prognosis in these patients. Pathogenesis of this syndrome remains unknown, despite demonstration in one case of a possible influence of zinc deficiency.