Ainhum

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.

Dactylosis Spontanea.

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 this condition.

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 mutilating keratoderma.

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.