The use of an elevated aldolase in diagnosing and managing eosinophilic fasciitis.

Eosinophilic fasciitis (EF) is a rare localized fibrosing disorder of the fascia whose diagnosis is often suspected based on clinical findings and laboratory values. These lab abnormalities can be transient in early disease and may not always be present. We have reviewed a case series of patients to assess the utility of the various laboratory abnormalities in diagnosing EF. We performed a retrospective review of EF patients seen at Georgetown University Hospital in the Division of Rheumatology during 2009 and 2013. This review included 15 adult patients with EF with a mean age at diagnosis of 45 years (range 18 to 77 years). The majority of patients 13/15 had classic skin thickening documented on all four extremities Only eight patients had peripheral eosinophilia ranging between 8 and 38 %. In these patients, the peripheral eosinophilia was an early but transient finding. Inflammatory markers including the erythrocyte sedimentation rate (ESR) was elevated in 5/14 and C-reactive Protein (CRP) was elevated in 7/11. At disease presentation, only one of eleven patients checked had an elevated creatine phosphokinase (CPK). Aldolase levels were available for 12 of the 15 patients, and they were increased in 11 out of 12 patients. We have found that in this case series, aldolase was more likely to be abnormal than peripheral eosinophilia, hypergammaglobulinemia, and ESR particularly after starting treatment. Aldolase should be measured in all patients suspected of having EF, and may also play a useful role in following disease activity.

Scleroderma mimics.

Scleroderma is a rare systemic autoimmune disease with multiple organ manifestations, including skin fibrosis. The groups of disorders classified as scleroderma mimics share the common thread of skin thickening but are otherwise quite incongruous in terms of underlying disease process and other organ involvement. This article reviews the clinical presentation, etiology, and treatment options available for scleroderma mimics, including morphea, scleredema, diabetic cheiroarthropathy, scleromyxedema, nephrogenic systemic fibrosis, and eosinophilic fasciitis. Through greater understanding of these diseases and the associated extradermal implications, we hope to facilitate recognition of scleroderma and its mimics.