A rare paraneoplastic disorder.
Associated with malignancies of the ovary, lung, pancreas, bladder, colon, prostate, kidney, breast, fallopian tube, stomach, cervix, liver, chronic myelogenous leukemia, multiple myeloma, and Hodgkin’s disease.
Mainly affects women with rheumatologic symptoms, often triggering detection of an occult malignant process.
Differential diagnosis includes rheumatoid arthritis, scleroderma, reflex sympathetic dystrophy, Dupuytren’s contraction, diabetic arthropathy, and eosinophilic fasciitis.
No specific pathologenic mechanism has been elucidated and no specific diagnostic or radiographic tests are available.
Propose explanations include the presence of a fibroblast proliferative factor, activation of profibrotic factors, hormonal changes.
Associated with poor prognostic implications.
Treatment is for the underlying malignancy and often includes corticosteroids and chemotherapy.