ABSTRACT
Eosinophilic fasciitis (EF) is a rare scleroderma-like syndrome. There has been no previous published report of EF in a Thai patient. We described a 41 year-old Thai man who presented with symmetric induration of the skin of forearms, arms, hands, fingers, lower aspects of the legs, and feet. Physical examination revealed bilateral symmetrical woody induration of the skin with peau d’orange appearance. A groove sign was positive on the flexural surface of both arms. Laboratory testing revealed a peripheral eosinophil count of 54%. The skin and superficial fascia biopsy specimen from the inner aspect of the left forearm was consistent with EF. He was treated with prednisolone, methotrexate, and colchicine. He experienced a gradual improvement within 4 months. A history of acute onset of scleroderma-like syndrome and careful physical examination can lead us to the diagnosis of EF.
ABSTRACT
Hyper-IgE syndrome (HIES) is a rare idiopathic primary immunodeficiency. It is characterized by a triad of findings, including high levels of serum IgE, recurrent skin abscesses and pneumonia and leads to pneuma-tocele formation. The diagnosis of HIES is complicated by a diversity of clinical and immunological spectrums and a heterogeneous set of genetic defects. The National Institute of Health (NIH) developed a scoring system for HIES in which a score greater than 14 indicates a probable diagnosis. Our patient presented with recurrent multiple ab-scesses on her scalp, recalcitrant eczema, candida onychomycosis, alopecia universalis, and highly elevated levels of serum IgE. Using the NIH scoring system, a 30 total-point score in this patient indicated the likelihood of carrying the HIES genotype. To our knowledge, there are no specific treatments of HIES. The humanized recombinant mo-noclonal antibody against IgE, subcutaneous omalizumab, was successfully used in this patient.