eosinophilic fasciitis (Shulman's syndrome)
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Introduction
A scleroderma spectrum disorder[2]
Etiology
- associated conditions
- antibody-mediated aplastic anemia & thrombocytopenia
- leukemia
- myeloproliferative disease
- Hodgkin's disease
Epidemiology
- affects men & women equal
- most commonly presents in 4th & 5th decades of life
Pathology
- involves the fascia with relative sparing of dermis & epidermis
- perivascular mixed inflammatory cell infiltrate in muscle
- lymphocytes, plasma cells & eosinophils infiltrating deep fascia[2]
- no systemic involvement[2]
Clinical manifestations
- tight skin in the extremities, sparing the face, hands & feet
- skin on hand should wrinkle with pressure in contrast to scleroderma or CREST syndrome
- woody induration of the skin
- does NOT cause Raynaud's phenomenon
- sunken veins due to skin retraction may be seen when an extremity is elevated (groove sign)
- orange skin changes may occur
- onset after vigorous exercise is common
- flexion contractures may develop
- carpal tunnel syndrome may develop
- arthralgias
- arthritis (40%)
- no visceral involvement
- lesions are painful[2]
Laboratory
- complete blood count with differential
- eosinophilia (70%)
- thrombocytopenia &/or anemia (occasionally)
- increased erythrocyte sedimentation rate (ESR)
- hypergammaglobulinemia
- full thickness skin biopsy, punch biopsy
- thickening & inflammation of t deep fascia with eosinophil infiltration
- antinuclear antibodies may be positive or negative
Complications
- blood dyscrasias (10%)
- flexion contractures
- carpal tunnel syndrome
Differential diagnosis
- scleroderma or CREST syndrome
- associated with scleroderma autoantibodies, specifically anticentromere antibody, thus anti-nuclear antibody positive
- widespread skin fibrosis, often involving the fingers (sclerodactyly), Raynaud phenomenon
- morphea plaques[2]
- occur on the face, head, neck, trunk (especially around the belt line), & lower extremities
- usually oval & initially are indurated, with a yellow to brown color
- when lesions are active, they are surrounded by a violaceous halo
- over time, plaques may appear more atrophic & less indurated[2]
- lesions may be mildly painful or painless
- eosinophilia myalgia syndrome
- generalized lichen sclerosus et atrophicus
- porphyria cutanea tarda
- chronic Lyme disease
Management
- prednisone 20-40 mg QD
- most effective treatment
- variable duration & efficacy of response
- methotrexate
- dermatology referral may be needed for biopsy & definitive diagnosis
- surgery make be necessary to reduce contractures
- prognosis:
- spontaneous regression after 2-5 years may occur
More general terms
Additional terms
- CREST syndrome
- eosinophilia-myalgia syndrome (EMS)
- scleroderma (diffuse cutaneous systemic sclerosis)
- toxic oil syndrome
References
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 177, 876
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16 17, 18. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018.
Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025 - ↑ Ferri's Clinical Advisor, Instant Diagnosis and Treatment, Ferri FF (ed), Mosby, Philadelphia, 2003, pg 305
- ↑ Boin F, Hummers LK. Scleroderma-like fibrosing disorders. Rheum Dis Clin North Am. 2008 Feb;34(1):199-220 PMID: https://pubmed.ncbi.nlm.nih.gov/18329541
- ↑ Mertens JS, Seyger MMB, Thurlings RM et al Morphea and Eosinophilic Fasciitis: An Update. Am J Clin Dermatol. 2017 Aug;18(4):491-512. PMID: https://pubmed.ncbi.nlm.nih.gov/28303481 Free PMC article. Review.
Patient information
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