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
Laboratory
- complete blood count with differential
- eosinophilia (70%)
- thrombocytopenia &/or anemia (occasionally)
- increased erythrocyte sedimentation rate (ESR)
- hypergammaglobulinemia
- full thickness skin biopsy, punch biopsy
Complications
- blood dyscrasias (10%)
- flexion contractures
- carpal tunnel syndrome
Differential diagnosis
- scleroderma or CREST syndrome
- 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 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16 17, 18. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018.
- ↑ 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://www.ncbi.nlm.nih.gov/pubmed/18329541
Patient information
eosinophilic fasciitis patient information