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

ABSTRACT: 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.

SUBMITTER: Thonnes S

PROVIDER: S-EPMC6754008 | biostudies-other | 2017 Mar

REPOSITORIES: biostudies-other

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

Thönnes Simon S Sorg Heiko H Hauser Jörg J Tilkorn Daniel J DJ

Innovative surgical sciences 20170307 1

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 ...[more]

PMID: 31579730

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Project description:Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. The exact driving mechanisms behind morphea and EF pathogenesis remain to be elucidated. However, extensive extracellular matrix formation and autoimmune dysfunction are thought to be key pathogenic processes. Likewise, these processes are considered essential in systemic sclerosis (SSc) pathogenesis. In addition, similarities in clinical presentation between morphea and SSc have led to many theories about their relatedness. Importantly, morphea may be differentiated from SSc based on absence of sclerodactyly, Raynaud's phenomenon, and nailfold capillary changes. The diagnosis of morphea is often based on characteristic clinical findings. Histopathological evaluation of skin biopsies and laboratory tests are not necessary in the majority of morphea cases. However, full-thickness skin biopsies, containing fascia and muscle tissue, are required for the diagnosis of EF. Monitoring of disease activity and damage, especially of subcutaneous involvement, is one of the most challenging aspects of morphea care. Therefore, data harmonization is crucial for optimizing standard care and for comparability of study results. Recently, the localized scleroderma cutaneous assessment tool (LoSCAT) has been developed and validated for morphea. The LoSCAT is currently the most widely reported outcome measure for morphea. Care providers should take disease subtype, degree of activity, depth of involvement, and quality-of-life impairments into account when initiating treatment. In most patients with circumscribed superficial subtypes, treatment with topical therapies suffices. In more widespread disease, UVA1 phototherapy or systemic treatment with methotrexate (MTX), with or without a systemic corticosteroid combination, should be initiated. Disappointingly, few alternatives for MTX have been described and additional research is still needed to optimize treatment for these debilitating conditions. In this review, we present a state-of-the-art flow chart that guides care providers in the treatment of morphea and EF.

Dataset Information

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

Publications

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

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