Destructive deformation of the digits with auto‐amputation: a review of pseudo‐ainhum

Abstract

Pseudo-ainhum is an auto-amputation of the digits. Although extremely rare, it is a traumatic and painful experience that can be alleviated with early recognition and intervention. The scientific literature is filled with reports of this interesting but unfortunate phenomenon. To date, a firm causative aetiology has not yet been established. Although reports on this phenomenon have attempted to further our understanding of pseudo-ainhum, a clear understanding has been complicated by the interchangeable use of terms describing this auto-amputation. In this review, we discuss the current understanding, diagnostic criteria, and management of pseudo-ainhum. Furthermore, the nomenclature of pseudo-ainhum is clarified. Ideally, this will allow for more efficient exploration of pseudo-ainhum, its causes, and therapeutic approaches.

Ainhum (Dactylolysis Spontanea): A Rare Cause of Consult in Dermatology- A Case Report and Review of Literature

“…The main differential diagnosis is with pseudo-ainhum, an auto-amputation of the digits that results of an identifiable or associated disease process and is independent of race. Two spectra of pseudo-ainhum has been described: the primary pseudo-ainhum, characterized by a congenital condition presented in the first ages [2] as a result of an identifiable or associated process; and the secondary pseudo-ainhum, characterized by presentation in later in life and associated with keratodermia also named Vohwinkel Syndrome [9], trauma, Hansen's disease, scleroderma, atypical keratoderma, syringomelia, syphilis, vascular or diabetic gangrene [3].…”

“…The term ainhum or Dactylolysis Spontanea was used for first time by da Silva Lima in 1867 [1,2]. It is believed that the word "ainhum" derives from the Brazilian word "Nago" meaning 'fissure' or the Nigerian word "Yoruba" meaning 'to saw' [1].…”

“…Ainhum or Dactylolysis Spontanea is used for describe African or African descendent patients with a clinical history of a groove or fissure of constricting tissue around the proximal end of the digits that affects mainly the fifth toe [1]. Meanwhile, pseudoainhum is any case of auto-amputation independent of race that may classify in two spectra `primary pseudo-ainhum' or 'secondary pseudo-ainhum' depending the age of presentation and the associated conditions [2]. We report an African American descendent adult with clinical history consisting with classic ainhum or Dactylolysis Spontanea.…”

Pseudoainhum numa Doente com Doença Mista do Tecido Conjuntivo: Uma Associação Incomum

“…Pseudoainhum, on the other hand, occurs as a result of an identifiable disease in patients of all ethnicities. 1,3 A detailed workup to rule out associated syndromes or underlying diseases should therefore be performed, considering the possibility of scleroderma, discoid lupus erythematous, KS (Vohwinkel´s syndrome and Mal de Meleda), psoriasis and Reynolds syndrome. 1,2,4,5 Due to this patient's positivity to anti-U1-RNP antibodies we considered a possible MCTD.…”

“…As opposed to ainhum (dactylolysis spontanea), an idiopathic band-like constriction that affects toes of patients in the underdeveloped Third World, pseudoainhum is the secondary form and may be associated with hereditary and nonhereditary disorders, connective tissue diseases (CTDs) and keratinization syndromes (KS). [1][2][3] We present a case of the previously unreported association, between pseudoainhum and mixed connective tissue disease (MCTD).…”