Surgical correction of pseudoainhum in chronic epidermolysis bullosa: a case report

Abstract

Pseudoainhum is a rare condition of unknown aetiology that is characterised by the presence of constriction bands of extremities due to a variety of underlying causes that progress to irreversible damage and auto-amputation of the affected digit. Epidermolysis bullosa is a group of disorders characterised by extremely fragile skin and recurrent blister formation resulting from minor mechanical trauma. The association of epidermolysis bullosa with other hand defects, such as hand contracture and pseudosyndactyly, have been reported; however, ainhum-like digital constriction bands have not been reported in association with this disorder. To our knowledge, this is the first reported case of pseudoainhum in association with epidermolysis bullosa and highlights good surgical response obtained with microscopic removal of the recurred constriction band and cross-finger flap to prevent recurrence, relieve symptoms and improve cosmesis.