‘Venous Furrowing’: A Clue to the Diagnosis of Eosinophilic Fasciitis

Horacek, E.; Sator, Paul-Gunther; Gschnait, F

doi:10.1159/000102041

Cited by 7 publications

(1 citation statement)

References 18 publications

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“…On the contrary, none of the described associated factors, such as arthropod-induced diseases (e.g. borreliosis), medication with statins or phenytoin, and severe disease of the thyroid or bone marrow (Table 1) [2], [4], were identified in our case. Laboratory diagnostics showed typical eosinophilia of the disease neither initially nor in the 6-month follow-up examination.…”

Section: Case Reportmentioning

confidence: 63%

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

Thönnes

Sorg

Hauser

et al. 2017

Innovative Surgical Sciences

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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.Keywords: eosinophilic fasciitis; nerve compression syndrome; peripheral nerve tumor; Shulman's disease. Case reportWe present the case of a 46-year-old man suffering from pain and dysesthesia over the ulnar nerve of his right arm for a duration of about 7 weeks. The presenting symptoms were tingling sensations in the lateral forearm and pain on palpation between the distal bicipital sulcus and the ulnar sulcus of the elbow. Electrodiagnostic examination showed a reduced nerve conduction velocity of 34.1 m/s over the affected cubital tunnel and 46 m/s distally in the forearm. The synopsis of the clinical signs leads to the patient's referral to our department with the diagnosis of nerve compression syndrome of the ulnar nerve.Clinical evaluation showed no muscle atrophy of the upper extremity but dysesthesia in the palmar area of the fourth and fifth fingers of his right hand. The pain over the ulnar nerve was triggered by palpation of the nerve between the distal bicipital sulcus and the ulnar sulcus of the elbow, where unexpectedly a subcutaneous tumor of 5 × 2 cm was palpable as a pathologic correlate. Magnetic resonance imaging (MRI) revealed a tumorous malformation of the ulnar nerve proximal to the ulnar groove matching the palpated size and most likely a schwannoma (Figure 1). This supported the indication for an exploration of the ulnar nerve due to the nerve compression, most likely caused by a peripheral nerve tumor.Surgical exploration demonstrated the ulnar nerve distended over a length of 5 cm, indurated and entrapped into an inflammatory tissue (Figure 2). The perineurium was dissected and a sample of one fascicle of the ulnar nerve was obtained. Histopath...

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Section: Case Reportmentioning

confidence: 63%

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

Thönnes

Sorg

Hauser

et al. 2017

Innovative Surgical Sciences

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The ‘Connective Tissue Diseases’

Goodfield¹,

Jones²,

Veale³

2010

Rook's Textbook of Dermatology

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Shulman-Syndrom (Eosinophile Fasziitis)

et al. 2009

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Eosinophilic fasciitis is a rare disease characterized by edema, painful indurations, and progressive muscle weakness. Mainly the extremities are involved. We report on a 22-year-old woman with eosinophilic fasciitis presenting with progressive muscle weakness of both hands and feet and a reduced general condition. She showed symmetrical and firm swelling of the extremities with painful restriction of joint movement. Systemic treatment with glucocorticosteroids as well as physiotherapy and manual lymphatic drainage led to continuous improvement of her symptoms. The differentiation from other diseases, such as systemic scleroderma, eosinophilia-myalgia syndrome, and pseudoscleroderma, might be difficult at the beginning of the disease. The gold standard for diagnosis is--as was done in our case--a deep skin-to-muscle biopsy. Further imaging, especially magnetic resonance imaging, can support the diagnostic procedure.

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‘Venous Furrowing’: A Clue to the Diagnosis of Eosinophilic Fasciitis

Cited by 7 publications

References 18 publications

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

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

The ‘Connective Tissue Diseases’

Shulman-Syndrom (Eosinophile Fasziitis)

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