Bazex syndrome revealing a gastric cancer.

We herein report the case of a 73-year-old woman who developed skin and nail disorders 2 months before her digestive symptoms started, which lead to the diagnosis of gastric adenocarcinoma. The lesions were diagnosed as Bazex syndrome, usually seen in squamous cell carcinoma. Under systemic chemotherapy, the cutaneous signs improved for some months before worsening when the disease progressed.

Introduction

Bazex syndrome or acrokeratosis paraneoplastica is a rare paraneoplastic syndrome that is characterized by acral psoriasiform lesions [1]. This condition is a distinct skin phenomenon and can represent the first sign of a supra-diaphragmatic neoplasia, usually squamous cell carcinoma [2]. Patients typically present with asymptomatic and symmetrical acral erythemato-squamous psoriasiform eruptions involving fingers, hands, nails, ears, nose, and feet [3]. The histological findings are not specific but include psoriasiform epidermal hyperplasia, hyperkeratosis with parakeratosis, and perivascular lymphocytic infiltrates in the dermis. Here, we report a case of a 73-year-old woman with Bazex syndrome, which was the first sign of a gastric cancer (linitis plastica).

Case Report

A 73-year-old woman presented with symptoms of food aversion and dysphagia (grade III). Her medical history consisted of tobacco smoking and iliac angioplasty. She had no prior history of psoriasis or other skin diseases. However, 2 months before the first digestive sign, she developed nail and finger abnormalities that predominantly affected her left hand, but it also involved the right hand and her feet. She had lost 7 kg within 3 months and weighed 38 kg at the time of the consultation. Her clinical examination was normal, except for the skin and nail lesions (fig. 1). She underwent an upper endoscopy and endoscopic ultrasound with biopsies that led to the diagnosis of gastric linitis, extending from the gastroesophageal junction to the antrum. She was also diagnosed with ovarian metastases after a CT scan. A FOLFOX4 chemotherapy regimen was then given every 2 weeks. Her general status improved after 1 month of systemic chemotherapy, including an improvement of the Bazex syndrome. This response lasted 6 months; after that, her skin lesions, anorexia, and her performance status all worsened, and the development of ascites was detected. Despite second-line treatment with a FOLFIRI chemotherapy regimen, the outcome was fatal 10 months after her initial diagnosis.

Fig. 1

Right-hand picture showing skin and nail lesions that are typical of Bazex syndrome.

Discussion

Bazex syndrome is an infrequent paraneoplastic marker [4], but it is important to recognize its appearance. In a majority of cases, cutaneous findings precede the onset of symptoms of an underlying neoplasm by several months. The implicated cancers are most often squamous cell carcinomas involving the upper aerodigestive tract. Cases associated with gastric adenocarcinoma are very rare. The pathogenesis of Bazex syndrome remains unknown. It may be caused by the production of epidermal growth factor by tumor cells or by cross-reactivity between epidermal and tumor antigens [5]. In approximately 90% of all cases, the dermatosis follows the neoplastic course with an improvement after the effective treatment of the neoplasia [6] and a recurrence when the tumor returns. Diagnosis is based on clinical and histological findings. A complete evaluation of the upper aerodigestive tract should be performed to identify the underlying malignancy.

Disclosure Statement

The authors declare no conflict of interest.