Introduction Demographics Causes Clinical features Complications Diagnosis Differential diagnoses Treatment Outcome
Eosinophilic fasciitis, also called Shulman syndrome, is a rare variant of scleroderma or deep form of morphoea involving the subcutis and fascia of the skin characterised by fascial thickening with an eosinophilic tissue infiltrate and peripheral eosinophilia.
Eosinophilic fasciitis usually affects adults aged 20–60 years, although there are reports in children and the elderly. Both sexes can be affected, with a slight male predominance (1.5:1). Occurrence in siblings has been reported but it is not regarded as an inherited condition. It may predominantly affect Caucasians, although there are cases reported from Japan, India, and other patients with skin of colour.
Eosinophilic fasciitis is possibly the result of an autoimmune mechanism targeting damaged fascia. Autoantibodies including rheumatoid factor (RF) and antinuclear antibodies (ANA) are sometimes detected.
Eosinophils may release transforming growth factor (TGF) beta to activate fibroblasts resulting in increased expression of type I collagen, fibronectin, and tissue inhibitor of metalloproteinase-1 (TMIP-1) resulting in fibrosis.
Triggers for developing eosinophilic fasciitis include:
Eosinophilic fasciitis typically develops suddenly 1–2 weeks after strenuous exercise or work; less commonly the onset is gradual.
Image of groove sign in eosinophilic fasciitis: Oliveira M, Patinha F, Marinho A. Eosinophilic fasciitis after parasite infection. Reumatologia. 2016;54(1):38-41. doi:10.5114/reum.2016.58761 Journal
Image of peau d'orange in eosinophilic fasciitis: Adachi Y, Mizutani Y, Shu E, Kanoh H, Miyazaki T, Seishima M. Eosinophilic fasciitis associated with myositis. Case Rep Dermatol. 2015;7(1):79-83. doi:10.1159/000381845 Journal
Eosinophilic fasciitis should be considered on the typical history and examination, and confirmed on deep skin biopsy including subcutis, fascia, and muscle [see Eosinophilic fasciitis pathology].
Diagnosis of eosinophilic fasciitis requires the major criterion and one or both of the two minor criteria:
Blood tests in early active disease:
Eosinophilic fasciitis can be associated with or progress to morphoea or other autoimmune connective tissue disorders in 20–30% of cases. Associated haematological disorders such as aplastic anaemia and lymphoma have been reported.
Spontaneous recovery is reported in 10–20% after 2–5 years. Treatment is typically required for months to years. The prognosis for survival is good; joint contractures and skin hardness may persist if treatment is delayed.
