Clinical Image: Acro-osteolysis.

A 58‐year‐old female with an 18‐year history of limited cutaneous systemic sclerosis presented with severe pain and ulceration affecting the tips of her fingers. On examination, there was evidence of auto‐amputation of some of her distal phalanges [Panel A – 1, 2, and 3] and ulceration of the overlying skin. Hand radiographs demonstrated resorption of the first and fourth distal phalanges as well as the second, third, and fifth middle phalanges of the right hand, in addition to resorption of the first, second, fourth, and fifth distal phalanges as well as the third middle phalanx of the left hand (acro‐osteolysis), along with resorption of overlying soft tissues (Panel B). These changes had developed insidiously because of severe persistent digital ischemia associated with systemic sclerosis. She did not get maximum pain relief with trials of various oral vasodilators, such as calcium channel blockers, alpha‐blockers, losartan, pentoxifylline, phosphodiesterase‐5 inhibitors, and topical nitroglycerin. Intravenous prostaglandin analogs, eg, prostacyclin, alprostadil, and treprostinil, are useful for the treatment of severe digital ischemia in systemic sclerosis. Thus, she was admitted for a 5‐day course of continuous intravenous alprostadil infusion, which was chosen for its ease of use and lower price 1. It provided substantial pain relief lasting for several months.

Acro‐osteolysis is associated with a diverse group of illnesses, such as scleroderma spectrum disorders, psoriasis, hyperparathyroidism, diabetes mellitus, leprosy, thermal burns, frostbite, and chronic polyvinyl chloride exposure. It can also occur in rare genetic disorders such as progeria, pyknodysostosis, and Hajdu‐Cheney syndrome.

Acro‐osteolysis has been estimated to occur in about 20% to 25% of patients with systemic sclerosis 2, 3. It is believed to result primarily from hypoxia‐induced upregulation of vascular endothelial growth factor, leading to increased osteoclastogenesis and enhanced osteoclastic bone resorption 4. However, although acro‐osteolysis is assumed to result from critical digital ischemia, it often develops even without digital ulcers, indicating that other factors may also play an essential role in its pathogenesis 4.