Acrokeratosis paraneoplastica in a 45-yr-old man

presented by

Eric Ehrsam MD

Lille, France

February 20, 2008

Eric Ehrsam MD, Dermatologist, Cabinet de dermatologie, Le Cateau Cambresis, France

 
Abstract 45 yo man with 6 month history of hyperkeratosis on his fingers, toes and ears. Examination revealed 2 carcinomas: on left tonsil and on the oesophagus. This was diagnosed as Bazex Syndrome.
Patient
45-year-old man
Duration
6 months
Distribution
ears, hands and feet
History

A 45-year-old man consulted for a hyperkeratosis on his fingers, toes and ears.

Physical Examination

Hyperkeratosis localized on toes, fingers, and ears (helix). The physical exam also revealed a hard left cervical lymph node.

Images

 

Laboratory Data

blood counts and biochemistry normal

Histopathology

nil

Diagnosis

Bazex syndrome (Acrokeratosis Paraneoplastica)

Reasons Presented

The patient was referred to an ENT specialist. Examination revealed 2 carcinomas: squamous cell carcinoma of the left tonsil T2 N2b MO and squamous cell carcinoma of the esophagus

The diagnosis of Bazex Syndrome lead to find the cancer at the origin of this paraneoplastic syndrome.

Planned treatments: transmandibular pharyngectomy and curettage of cervical lymph nodes, radiotherapy on ENT area and esophageal area.

Questions What is your diagnosis?
References
  1. Richard M, Giroux JM: Acrokeratosis paraneoplastica (Bazex' syndrome). J Am Acad Dermatol 1987 Jan; 16(1 Pt 2): 178-83
  2. Valdivielso M, Longo I, Suárez R, Huerta M, Lázaro P. Acrokeratosis paraneoplastica: Bazex syndrome. J Eur Acad Dermatol Venereol. 2005 May;19(3):340-4. Review.
  3. Bazex A, Griffiths A. Acrokeratosis paraneoplastica--a new cutaneous marker of malignancy. Br J Dermatol. 1980 Sep;103(3):301-6. Review.
Keywords Bazex Syndrome, Acrokeratosis paraneoplastica
Comments from Faculty and Members

Henry Foong, FRCP (Edin), Consultant Dermatologist, Ipoh Specialist Hospital, Ipoh, Malaysia on February 20, 2008

Great teaching and classical case! Walter Shelley MD, PhD wrote a nice piece on Andre Bazex in his "Classics in Clinical Dermatology" .

Andre Bazex was born on February 14, 1911 in Montestruc, Sur Gers, France. Andre grew up in Auer as the son of the local physician who died when Andre was 14. Determined to follow in his father's footsteps he went to Toulouse for medical training and specialization in Dermatology under famed Professor Andre Nanta

By 1941 he was Director of the Venereological Department........Read on "Andre Bazex and Bazex Syndrome"

Amor Khachemoune, MD,Assistant Professor, Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NY, USA on February 20, 2008

Yes, agree. Well documented case.

Ian McColl MBChB, FACD, Consultant Dermatologist, John Flynn Medical Centre, Tugun, Queensland, Australia on February 20, 2008

Great convincing images of a rare condition but with the malignancy in the classical upper airways or digestive tract and with a cervical node. They dont come more classical than this case!

Robert Rudolph MD, FACP, Clinical Professor of Dermatology, University of Pennsylvania, Philadelphia, PA, USA on Feb 20, 2008

Excellent case, and very instructive.

Question: If the node had not been present, would you still have considered Bazex based just on the ears and hands, with no changes in the nails or other psoriasiform lesions?

Congratulations on a "fine call".

Shahbaz Janjua MD, Dermatologist, Ayza Skin & Research Center, Lalamusa, Pakistan on February 20, 2008

It's a great case and the images are of very high quality. Congrats! I can also appreciate nail dystrophy (subungual hyperkeratosis and nail flaking) in the toe nails. What was the duration between the appearance of cutaneous lesions and the diagnosis of associated internal malignancies in this case?

Nico Mousdicas M.D., Associate Professor, Department of Dermatology, Indiana University, Indianapolis, indiana, USA on Feb 21, 2008

Thanks for sharing a great case. Great pictures too!

Devinder Mohan Thappa M.D., Professor and Head, Dermatology and STD Department, JIPMER, Pondicherry, India on Feb 21, 2008

Excellent, concise and illustrative presentation

Update: Eric Ehrsam MD, Dermatologist, Lille, France on Feb 21, 2008

The patient only consulted me because he complained of pains on his fingers. He did not have dysphagia or other symptoms. The left lymph node on the picture was apparent only when the patient turn his face to the right. He was a heavy smoker. I suspected the diagnosis at first glance and the presence of the lymph node was another main argument.

Arash Abtahian MD, Resident, Shiraz, Iran on Feb 22, 2008

The patient had had every thing for the this diagnosis and it has been the classical case of acrokeratosis paraneoplastica of Bazex.

Abbas Alshammari MD, Consultant dermatologist, Qatar Armed Forces Clinic, Doha, Qatar on Feb 22, 2008

Interesting and instructive case. Odd cases should be evaluated systemically with proper history and physical examination. Creative dermatologist should be good internist.

Omid Zargari MD, Rasht, Iran on Feb 22, 2008

Great case with excellent images. There are still many unanswered questions about paraneoplastic dermatoses.

For example, why the majority of cases of Bazex syndrome are men? Why is it connected to SCC rather than other types of carcinomas?

As I am interested in skin diseases of the nose, I'm very much interested to see a picture of nose involvement (if any) in this case.

Merci pour le partage, docteur Ehrsam!

Stelios Minas MD, Consultant Dermatologist, Limassol, Cyprus on Feb 23, 2008

Excellent presentation. Typical case of Bazex syndome. I just want to add one point. In my little practice I see some cases of Bazex syndrome with sign of Leser-Trélat. How often do you see this combination?

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