Please login or create an account. If you do not have access to this content, you will be shown a 30 second preview and licensing options.
- Presentation
Distinguishing Eosinophilic Fasciitis from Systemic Sclerosis and Silent Sinus Syndrome from Parry-Romberg Syndrome
Description
The talk focused on distinguishing two commonly confused dermatologic conditions using careful exam and targeted imaging. First, a 52-year-old man referred for suspected systemic sclerosis was instead diagnosed with eosinophilic fasciitis because he lacked distal digit involvement, Raynaud’s, and nailfold capillary changes, but had classic findings such as groove sign, peau d’orange, and a seatbelt sign. The speaker reviewed diagnosis with MRI or fascial biopsy, noted frequent eosinophilia and occasional monoclonal gammopathy, and discussed treatment options including systemic steroids, methotrexate, mycophenolate mofetil, and IVIG, with better outcomes when steroid-sparing agents are added. Second, a woman with lifelong facial asymmetry was initially considered for Parry-Romberg syndrome, but imaging showed congenital right facial bone hypoplasia without soft tissue atrophy and a hypoplastic, opacified maxillary sinus, confirming silent sinus syndrome. The speaker emphasized that silent sinus syndrome causes progressive facial asymmetry, hypoglobus, enophthalmos, and cheek depression due to chronic silent sinus inflammation and orbital floor collapse, and that imaging is essential to distinguish it from Parry-Romberg syndrome, which features soft tissue atrophy.
View moreConclusions
- Eosinophilic fasciitis can often be distinguished from systemic sclerosis by exam findings such as sparing of the distal digits, normal nailfolds, and absence of Raynaud’s phenomenon.
- MRI with contrast appears to be about as sensitive as fascial biopsy for confirming eosinophilic fasciitis and may be a useful less invasive diagnostic tool.
- Peripheral eosinophilia is common in eosinophilic fasciitis, but monoclonal gammopathy is usually not associated with later hematologic malignancy.
- Systemic corticosteroids alone produce complete remission in only a minority of eosinophilic fasciitis patients.
- Adding methotrexate or mycophenolate to steroids improves response rates and helps many patients taper off steroids.
- IVIG may be especially effective for refractory eosinophilic fasciitis, with high partial or complete response rates and steroid discontinuation.
- Silent sinus syndrome is an important non-inflammatory cause of progressive adult facial asymmetry that can mimic Parry-Romberg syndrome.
- Imaging is crucial for distinguishing silent sinus syndrome from Parry-Romberg syndrome because silent sinus syndrome shows hypoplastic maxillary sinus without soft tissue atrophy, whereas Parry-Romberg shows soft-tissue loss.
- Treatment of silent sinus syndrome is primarily sinus surgery to prevent further progression, while residual asymmetry may need reconstructive procedures rather than medical therapy.
- Mazori DR, et al. Curr Rheumatol Rep. 2017;19(12):74.
- Characteristics and Outcomes of Eosinophilic Fasciitis-Associated Monoclonal Gammopathy. JAMA Dermatology. 2021;157(12):1508-1509. doi:10.1001/jamadermatol.2021.4302.#10.1001/jamadermatol.2021.4302
- Wright NA, Mazori DR, et al. JAMA Dermatol. 2016;152(1):97-99.
- Mycophenolate Mofetil for Eosinophilic Fasciitis: A Retrospective Analysis From 3 Tertiary Care Centers. JAMA Dermatology.#10.1001/jamadermatol.2020.0120
- Intravenous immunoglobulin for refractory eosinophilic fasciitis: A retrospective analysis from 3 tertiary care centers.#10.1016/j.jaad.2019.12.022
- Dermarkarian CP, et al. Int Ophthalmol. 2021;41(2):599-604.
- Burroughs JR, et al. Ophthalmic Plast Reconstr Surg. 2003;19(6):449-54.