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

 

Chapter from W Shelley and J Crissey "Classics in Clinical Dermatology with biographical sketches 50th Annivesary" 2nd Edition. 2007

Andre Bazex

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 Adre 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. His distinguished career continued with his appointment in 1954 as Chairman and Professor of Dermatology at the University of Toulouse. His numerous publications included the description of unique cutaneous changes resulting from underlying cancer, as well as a distinctive inherited atrophy of the hair follicles. They are referred to as Bazex Syndrome and Bazex Atrophoderma respectively. He went on to become the President of the Societe Francaise de Dermatologie and to honorary membership in many foreign dermatologic societies.

Curiosities was his passion and his remarkable histopathologic and clinical skills made for exciting Grand Rounds. His fellow professors in internal medicine, anatomy, and biochemistry considered themselves privileged to join him in search for knowledge of skin disease. Students came from all over Europe to his clinic in Toulouse inspired by this modest, reserved, hard-working leader. And his many honors and medals attested to his scientific productivity.

In 1980 Bazex retired enjoying philosophic discussion groups as well as intensive study of the World War II. His death on October 18, 1988 left dermatology a treasured heritage: distinguished Professor of Dermatology, his son, Jacques Bazex.

 

Bazex Syndrome

Syndrome Para-Neoplasique A Type D'hyperkeratose Des

Extremities: Guerison apres le traitement de l'epithelioma larynge*

Mr C. Jean Marie, 60-years-old was referred to us by his doctor January 13 for hyperkeratotic lesions of the extremities of fairly recent onset. To be frank, the patient has a low I.Q. and it is difficult to obtain an exact history. The lesions are polymorphic: plantar keratoderma appearing on the dorsum of the toes, on the palms and on the back of the fingers, dry follicular parakeratosis of the nose,ichthyotic changes on the face and ears, and pseudo-tinea amiantacea of the scalp. This ensemble brings to mind pityriasia rubra pilaris, a localized ichthyosiform process or a seborreic dermatitis.

Physical examination of the patient reveals the presence of submaxillary and jugular adenopathy, which permits us to discern the existance of a neoplasm in the buccal -laryngeal area that only hoarseness would have led us to suspect. It proved to be a very advanced epithelioma, possibly only responsive to palliative cobalt therapy, preceded by local injections by endoxan and methotrexate in the superior thyroid artery.

From the initiation of this treatment and parallel improvement of the epithelioma, the keratotic lesions cleared and presently the hands and face are entirely cured.

This case therefore meets the criteria to be considered as a paraneoplastic dermatoses as delineated by the Bureau and H.O Curth of the XI Congress of the Associations of the French Dermatologists. In effect, we note in the care of this patient, the almost simultaneous onset of the dermatoses and the neoplasm as well as the parallel evolution of the dermatoses and the neoplastic lesion. We can even speak here of it being the marker dermatoses.

This report can be compared with but is not identical to 1) palmar-plantar keratosis associated with epithelioma of the upper esophagus reported by Howel-Evans (palmar and plantar tylosis) and 2) the ichthyosiform changes accompanying palmar -planar hyperkeratosis reported by Glasebrook)

* Bull.Soc. Fr. Dermatol Syphil. 72-82, 1965

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