HPI

• 57-year-old female presents with 2.5 months of bilateral lower extremity erythema, pain, and swelling
• Symptoms have been constant and worsening throughout this time
• Initially isolated to the ankle but has extended to the groin on the posterior aspect of the legs
• Pt has completed a course of both Keflex and Clindamycin with no improvement

Exam

VS: T 100.7 HR 115

• Bilateral lower extremities appear like this

Clinical Course

• Pt is started on broad spectrum IV antibiotics
• Duplex US of LE are negative
• ESR 40, CRP 1.17, WBC 3.78, AST 40
• Patient requires multiple doses of pain medications and is admitted for pain control and further evaluation
• Inpatient further lab testing included RPR, C3/C4, Rheumatoid Factor, SPEP, anti-CCP, ANA, all of which were normal
• MRI of the RLE shows subcutaneous soft tissue infiltration
• Fascial biopsy is performed by Dermatology and shows: Eosinophilic Fasciitis 

Eosinophilic Fasciitis aka Shulman Syndrome

• Characterized by limb edema and erythema that transitions into thickening of underlying fascia
• Etiology is often unknown but it is occasionally associated with Borrelia infections, certain medications (i.e. phenytoin), new dialysis, autoimmune diseases, hematologic disorders (i.e. lymphoma, multiple myeloma, aplastic anemia, etc.)
• Almost always bilateral and symmetric, spares hands and feet
• Develops into peau de’orange texture
• Associated symptoms include inflammatory arthritis, myalgias, neuropathy
• Diagnosis: Typically have elevated ESR and CRP, MRI helps rule out differential diagnosis, biopsy is required for definitive diagnosis
• Treatment: Long term oral steroids- typically starting prednisone 1mg/kg until symptoms start to resolve then can taper off If no improvement on steroids patients may ultimately require immunotherapy such as methotrexate, hydroxychloroquine, mycophenolate, or monoclonal antibodies
• Differential Diagnosis Include: Scleroderma- like diseases: Lipo-dermatosclerosis Scleromyxedema Localized scleroderma Morphea Cutaneous Sarcoid Systemic Sclerosis Graft versus Host Disease

References:

Eosinophilic fasciits. (2015, August 8). Retrieved January 12, 2021, from http://www.pcds.org.uk/clinical-guidance/eosinophilic-fasciits Long, H., Zhang, G., Wang, L., & Lu, Q. (2015).

Eosinophilic Skin Diseases: A Comprehensive Review. Clinical Reviews in Allergy & Immunology, 50(2), 189-213. doi:10.1007/s12016-015-8485-8 Pinal-Fernandez, I., Callaghan, A. S., & Grau, J. (2014).

Diagnosis and classification of eosinophilic fasciitis. Autoimmunity Reviews, 13(4-5), 379-382. doi:10.1016/j.autrev.2014.01.019