When the woman was admitted, the differential diagnosis was between scleroderma and eosinophilic fasciitis (also known as generalized morphea profunda), according to Dr. Irwin M. Braverman, who presented the case at the Fall Clinical Dermatology Conference.
The woman had plaques of morphea covering her entire body. The edema also affected her breasts but spared the areolae and nipples. She had large plaques on her abdomen. Histology showed deep dermal fibrosis with fibrosis of fat septa extending down to a thickened fascial layer, which is confirmatory for eosinophilic fasciitis, said Dr. Braverman, professor of dermatology at Yale University, New Haven, Conn.
“We have seen about a half-dozen patients with this syndrome in the last 4 years,” he added. “This starts suddenly with patients coming in with tender, painful swelling of the arms, legs, and trunk.”
The woman was started on 20 mg prednisone t.i.d. and tapered to 15 mg once a day over the course of 12 months. The dose was then reduced by 2.5 mg every 6 months. The regimen yielded a prompt decrease in induration, swelling, and pain.
She is currently taking 5 mg prednisone once a day, and her skin has returned almost to normal.
“She will continue to be tapered 2.5 mg every 8 weeks if she shows continued improvement,” Dr. Braverman said at the meeting, which was sponsored by the Center for Bio-Medical Communications, Inc.
He emphasized that eosinophilic fasciitis “is very responsive to steroids. After starting oral prednisone, within a week this woman was feeling no pain and the edema had subsided. It's taken almost 4 years, but over the course of a slow taper, her skin has returned essentially to normal.”
The steroid therapy regimen for eosinophilic fasciitis that has been most effective for his patients is as follows:
Start with 60 mg/day (20 mg t.i.d.) prednisone for 2 weeks tapering to 25 mg per day (10/10/5 mg) over 6 weeks.
Taper 2.5 mg every other week until you reach 15 mg/day (5 mg t.i.d.).
Taper 2.5 mg every 4 weeks (t.i.d. dosing) until you reach 10 mg/day.
Remain on 10 mg/day in a single morning dose for 2 months.
Taper 2.5 mg every 8 weeks.