Case Report
Bazex syndrome (acrokeratosis paraneoplastica) Brian Poligone, Sean R Christensen, Rossitza Lazova, Peter W Heald Lancet 2007; 369: 530 Department of Dermatology (B Poligone MD, S R Christensen PhD, R Lazova, MD), Yale University School of Medicine, New Haven, CT 06520, USA; and Dermatology Service (Prof P Heald MD), West Haven Veteran’s Administration Hospital West Haven, CT 06516, USA Correspondence to: Prof Peter W Heald
[email protected]
In March, 2006, a 62-year-old black man presented to his primary care physician with a 4-day history of bilateral auricular pruritus associated with pain. He had temporary improvement with topical triamcinolone and oral cefalexin. After 2 months of gradual worsening with involvement of the distal fingers and toes he was referred to us in May, 2006, for dermatological evaluation. Other than one upper respiratory infection he denied fevers, chills, or other systemic complaints. He had no dysphagia, odynophagia, change in appetite, weight loss, nausea, vomiting, altered bowel habits, haematochezia, or melaena. Medical history showed hypertension, a non-ischaemic cardiomyopathy (with an ejection fraction of 35%), and, 6 years previously, pulmonary tuberculosis, for which he had received appropriate antibiotic therapy. Current medications were aspirin, furosemide, metoprolol, lisinopril, and a salbutamol/ipratropium inhaler. He had a history of alcohol misuse and was currently drinking over half a litre of vodka per day. He had smoked 10 cigarettes a day for 50 years. On examination, there were hyperpigmented, erythematous plaques with silvery scales on the helices of both ears, and the dorsal surfaces of the metacarpophalangeal, proximal interphalangeal, and distal interphalangeal joints of the hands and feet which had only minor improvement with topical corticosteroids (figure A and B). Nail thickening with ridging was present. No oral or ocular lesions were noted. There was no heliotrope erythema, periungal telangiectasia, or poikyloderma. Cardiovascular, respiratory, and abdominal examinations were normal. No lymphadenopathy was detected. Bazex syndrome (acrokeratosis paraneoplastica) was suspected and chest radiography and upper gastrointestinal evaluation was requested. Skin biopsy showed acanthosis, psoriasiform epidermal hyperplasia, hyperkeratosis, focal parakeratosis,
Figure: Skin and nail changes on the hands (A) and feet (B)
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exocytosis, spongiosis, and spongiotic vesicles. There were superficial perivascular lymphocytic infiltrates and necrotic keratinocytes at the dermoepidermal junction. Barium swallow showed a broad based ulceration in the distal oesophagus, and follow-up CT scan and endoscopy showed invasive squamous cell carcinoma of the oesophagus (stage III). The patient was started on localised radiation therapy with adjuvant 5-fluorouracil and cisplatin. When seen for his second course of chemotherapy in June, 2006, a striking resolution of the hyperkeratosis on his hands and feet was noted. His ears had returned to normal with no more pruritus. The association of acral hyperkeratosis and malignancy was first described by Bazex in 1965.1 Bazex syndrome generally affects men over the age of 40 years and has universally been associated with malignancy in over 125 case reports. The most common sites of involvement are the ears (79%), nails (75%), nose (63%), fingers (61%), hands (57%), and feet (50%).2 The presenting symptoms in this patient, pruritus and pain, are not usually associated with Bazex syndrome,3 although pruritus can occur in up to 18% of cases.2 A wide range of histological findings has been described in Bazex syndrome, including acanthosis, psoriasiform epidermal hyperplasia, hyperkeratosis, parakeratosis, and perivascular lymphocytic infiltrate. Less common but more specific findings include dyskeratotic keratinocytes and vacuolar degeneration of the basal epidermal layer. More than half of acrokeratosis paraneoplastica associated malignancies are found in the upper aerodigestive tract (upper parts of the respiratory and gastrointestinal tracts). Regional lymphadenopathy is often present. In nearly two-thirds of cases, cutaneous lesions precede the symptoms or diagnosis of malignancy.2 Thus, suspicion of Bazex syndrome should include a skin biopsy followed by a complete evaluation of the upper aerodigestive tract. If this is negative, a search for the other malignancies reported with Bazex syndrome would have to include screening for colon cancer, lymphoma, bladder, prostate, uterine, vulvar, and neuroendocrine tumours.4 References 1 Bazex A, Salvador R, Dupre A, Christol B. Syndrome paranéoplasique à type d’hyperkératose des extrémités. Bull Soc Fr Dermatol Syphiligr 1965; 72: 182. 2 Bolognia JL, Brewer YP, Cooper DL. Bazex syndrome (acrokeratosis paraneoplastica). An analytic review. Medicine (Baltimore) 1991; 70: 269–80. 3 Richard M, Giroux JM. Acrokeratosis paraneoplastica (Bazex’ syndrome). J Am Acad Dermatol 1987; 16: 178–83. 4 Valdivielso M, Longo I, Suárez R, Huerta M, Lázaro P. Acrokeratosis paraneoplastic (Bazex Syndrome). JEADV 2005; 19: 340–44.
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