A Case Report of the Rare Disease of Pseudoainhum at Kerman Afzalipour Hospital, Iran

Background & Aims: Pseudoainhum is a rare acquired or congenital disorder characterized by progressive development of a fibrotic band on a finger or toe until spontaneous autoamputation occurs. The various etiological hypotheses given for this disease include infectious (mycosis, mycobacteria), traumatic (plants), vascular (andysplasia), neurological (polyneuritis), and genetic (keratodermia) mechanisms. Ainhum predominantly affects black people living in west Africa, south America, and India. Case presentation: A 12 year old girl from Chahrigan Harirabad, a village near Kahnooj, Iran, with low socioeconomic status whose parents were not related, presented with autoamputated right foot toes. According to her mother she was in her usual state of good health until 1 year of age, when she developed a deep painless wound in the base of the fifth toe of the right foot. The depth of the wound progressed over time until it autoamputed. The same process happened to the 2nd, 3rd, and 4th toes of the same foot. On physical examination the pulse of the posterior tibialis artery was too weak and there was no detectable pulse of the dorsalis pedis artery. All examinations of toes of the left foot were normal. There was also a kyphoscoliosis toward the left side. The laboratory tests were normal; however, triphasic Doppler sonography showed a decreased velocity of blood flow in the distal part of the anterior tibialis artery. Conclusion: Due to the rarity of the disease and genetics being the most important contributing factor, we suggest a genetic study of this patient and her family members, regular follow up, and physical examination of the patient. We also suggest that with promotion of the level of hygiene and adequate education we can prevent further damage in this patient, as trauma and subsequent infection are the likely predisposing factors.