A narrow strip of hardened skin with constricting ring
formation on the little toe at the level of digitoplantar fold,
progressively leading to spontaneous amputation.
Familial occurrence with autosomal dominant
inheritance has been described, but many sporadic cases have been reported.
Ainhum is characterized by the occurrence of a
circular constriction band most often located at the root of the fifth toe,
rarely of a finger. As constriction progresses, the toe becomes disabled,
and spontaneous amputation finally results. The disorder seems to be more
frequent in Africa. Classic features of the affected area include
hyperkeratosis, chronic dermatitis, ligamental destruction, and finally
osteoporosis with cortical bone resorption. Although the origin of the
disease remains to be elucidated, mechanical and/or inflammatory causes have
been favored by many authors.
There are no specific considerations for
Pseudoainhum: Ainhum-like constriction bands that also may
finally result in amputation of a digit (finger or toe) have been described
in conjunction with neurogenic acroosteolysis, genodermatoses, and
Streeter Anomaly: Constricting amniotic bands leading to
amputation with scarring, distal syndactyly, cleft lip/palate, anencephaly,
encephalocele, hydrocephaly, omphalocele, and gastroschisis. Other internal
anomalies involve the heart, lungs, diaphragm, kidneys, and gonads.
Adams-Oliver Syndrome (AOS): Very rare inherited disorder
characterized by defects of the scalp associated with multiple scarred and
hairless areas that usually have dilated blood vessels directly under the
skin. Scalp defects are present at birth. The extremities are either short
(hypoplastic fingers and toes) or characterized by absent hands and lower
legs. Congenital heart defect must be ruled out.
Genakos JJ, Cocores JA, Terris A: Ainhum (dactylolysis spontanea). Report
of a bilateral case and literature review. J Am Podiatr Med Assoc
Simon KMB: Ainhum, a family disease. JAMA 76:560, 1921.
Warter A, Audouin J, Sekou H: [Spontaneous dactylolysis or ainhum.
Histopathologic study]. Ann Pathol