Acropathia ulcero-mutilans non-familiaris M89.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Acropathia ulcero-mutilans acquisita; Acropathia ulcero-mutilans et deformans pseudosyringomyelitica; Acropathia ulcero-mutilans et deformans pseudo-syringomyelitica; Acropathy non familial pseudosyringomyelitis ulcero-mutilating; Acropathy non familial syringomylie-like ulcero-mutilating; Bureau-Barrière syndrome; non-familial syringomyelia-like ulceromutilising acropathy; Syringomyelia-like ulceromutilating acropathy nonfamilial

History
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Bureau and Barrière, 1955

Definition
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Non-familiar neurotrophic ulcerations and mutating acroosteolyses of the feet and hands, occurring in the context of a polyneuropathy (usually of ethyltoxic origin).

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Etiopathogenesis
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Unknown, provoking factor: chronic alcoholism, probably multifactorial.

Manifestation
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Mainly men between the ages of 40 and 50.

Localization
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Feet, mostly symmetrical.

Clinical features
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Deep, painless ulcerations with a hyperkeratotic border wall (see Collerette cornée). Stocking-like polyneuropathy, often with dissociated sensory disturbances. Non-reactive osteolysis of the loaded forefoot. Loss of thermal sensitivity, hyperhidrosis, lack of Achilles tendon reflex. Elephantiasis-like tissue hypertrophy, oedematous and verrucous changes, pachydermia, frequent recurrent erysipelas.

Laboratory
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Anemia, dysproteinemia.

Differential diagnosis
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Therapy
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Treatment of accompanying diseases (e.g. alcoholism). Otherwise symptomatic in cooperation with internists and orthopaedic surgeons. Avoidance of permanent mechanical stimuli and pressure relief (orthopaedic footwear). In case of ulcers, early treatment of the wound according to its stage. In case of osseous destruction surgical intervention necessary.

Literature
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  1. Bureau Y, Barrière H (1955) Acropathies pseudo-syringo-myéliques des membres inférieurs. Essai d'interprétation nosographique. Sem Hôp Paris 31: 1419-1429
  2. Castle G, Castle D (1990) The Bureau-Barrière syndrome. Phlebol Proctol 19: 147-152
  3. Köhn FM et al (1991) Acroosteopathia ulceromutilans Bureau barrier. Act Dermatol 17: 39-42
  4. Preisz K et al (2002) Launois-Bensaude syndrome and Bureau-Barriere syndrome in a psoriatic patient: successful treatment with carbamazepine. Eur J Dermatol 12: 267-269
  5. Vanhooteghem O et al (1999) Acropathia ulcero-mutilans acquisita of the upper limbs. Br J Dermatol 140: 334-337
  6. Vogl A et al (2005) Skin and alcohol. J Dtsch Dermatol Ges 3: 788-790

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Last updated on: 29.10.2020