Acrodystrophic neuropathy of Bureau and Barriere in Sudanese patient - Single case report and Review.

Abstract

Eight years girl presented with dystrophic changes involving fingers, toes and nails for 5 years, with hyperkeratotic lesions around the shin, painless trophic ulcer with chronic osteomylitis in right foot for 3 years. A syndrome of painless unhealed punch out, necrotic ulcer in the plantar aspect ball of right foot, and another one in same place left foot, hypotrichosis, striate palmoplantar keratoderma, onychogryphosis, clubbing of fingers, , acro-osteolysis, and psoriasislike skin lesions, for 3 years duration. Our case here has been documented to be a mutilating palmoplantar Keratoderma. The case is histopathologically confirmed to show Keratinized tissue. The condition as mentioned is extremely rare, where misdiagnosed as Epidermolysis Bullosa Dystrophica.

Keywords: Acrodystrophic neuropathy of Bureau and Barriere, Ulcerative-mutilating acropathy, Bureau-Barriere syndrome, Sudan.

Sudanese Journal of Dermatology Vol. 5 (2) 2008: pp. 76-80