Localized eosinophilic fasciitis (Shulman’s disease) as a differential diagnosis of nerve compression syndrome

• Main Authors: Thönnes Simon, Sorg Heiko, Hauser Jörg, Tilkorn Daniel J.
• Format: Article
• Language: English
• Published: De Gruyter 2017-03-01
• Series: Innovative Surgical Science
• Subjects: eosinophilic fasciitis; nerve compression syndrome; peripheral nerve tumor; shulman’s disease
• Online Access: https://doi.org/10.1515/iss-2016-0203

A 46-year-old man presented with clinical signs of nerve compression syndrome of his right ulnar nerve as confirmed by nerve conduction studies. Unexpectedly, clinical examination and magnetic resonance imaging (MRI) revealed a subcutaneous tumor of 5×2 cm above the ulnar groove. Surgical exploration and histopathology of biopsies demonstrated the nerve distended and entrapped into an eosinophilic, inflammatory tissue. This rare condition is consistent with localized eosinophilic fasciitis, with no systemic manifestations. There are reports of isolated forearm versions of the disease. However, none occurred with the entrapment of a peripheral nerve appearing as a peripheral nerve tumor, yet. Consequentially, the presented patient would not have benefitted from further surgical neurolysis or tumor debulking, as eosinophilic fasciitis is an inflammatory and systemic disease. The patient’s symptoms decreased spontaneously after 4 weeks of postsurgical treatment, including nonsteroidal anti-inflammatory drugs (NSAIDs). Altogether, this case proved the necessity to regard even rare diseases as a potential cause of entrapment of peripheral nerves. This should lead surgeons to critical, differential diagnostic thinking and suggest that systemic diseases may be encountered during surgery due to their capability to mimic peripheral nerve tumors.