ABSTRACT
Eosinophilic fasciitis (EF) is a rare systemic inflammatory disease with an unknown etiology. Making a diagnosis in such a case is always a challenge as it is a rare disease and mimics scleroderma and scleroderma-like syndrome but should be kept in mind as it carries a high mortality. Furthermore, it is a treatable disease. Here, we report a 41-year-old woman who presented to the rheumatology clinic at the Royal Hospital, Muscat, Oman, with a one-month history of bilateral swelling of the forearms along with skin tightness and fingers contraction. Her history and physical examination along with histopathological examination and magnetic resonance imaging findings were consistent with EF. She showed an excellent response to steroids and methotrexate which is not a combination therapy that has been tried or mentioned previously.
ABSTRACT
Hereditary angioedema (HAE) is a rare but serious and potentially life threatening autosomal dominant condition caused by low or dysfunctional C1 esterase inhibitor (C1-INH) or uncontrolled contact pathway activation. Symptoms are characterized by spontaneous, recurrent attacks of subcutaneous or submucosal swellings typically involving the face, tongue, larynx, extremities, genitalia or bowel. The prevalence of HAE is estimated to be 1:50,000 without known racial differences. It causes psychological stress as well as significant socioeconomic burden. Early treatment and prevention of attacks are associated with better patient outcome and lower socioeconomic burden. New treatments and a better evidence base for management are emerging which, together with a move from hospital-centered to patient-centered care, will enable individualized, tailored treatment approaches.
