[Good response of scleromyxedema and dermato-neuro syndrome to treatment with intravenous immunoglobulins].

نویسندگان

  • I Bielsa
  • F Benvenutti
  • R M Guinovart
  • C Ferrándiz
چکیده

Scleromyxedema is a potentially serious disease that can have various systemic complications. One of the most frequent forms of central nervous system involvement is dermato-neuro syndrome. High-dose intravenous immunoglobulins are among the drug treatments that have been used for this syndrome. We describe 2 patients with scleromyxedema, one of whom developed dermato-neuro syndrome. Both patients responded well to treatment with high-dose intravenous immunoglobulins. We suggest this therapy as a suitable first-line treatment for scleromyxedema and for its neurological complications.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Dermato-neuro syndrome in a case of scleromyxedema.

Scleromyxedema is an uncommon connective tissue disease characterized by mucin deposits, fibrosis, and proliferation of fibroblasts in the dermis. Although it shares similar sclerodermoid features, it is a different clinical entity than scleroderma. A monoclonal gammopathy is almost always present; however, progression to multiple myeloma is rare. It may have many systemic manifestations, of wh...

متن کامل

Treatment of scleromyxedema and the dermatoneuro syndrome with intravenous immunoglobulin.

Scleromyxedema is a rare disease characterized by extensive mucin deposition with fibrosis, and is associated with a monoclonal gammopathy. Currently there is no consensus on optimal treatment of this potentially fatal disease because of the lack of randomized controlled trials and limited number of case reports. At the time of this writing, 24 cases were published reporting clinical improvemen...

متن کامل

Suspected cardiac toxicity to intravenous immunoglobulin used for treatment of scleromyxedema.

Scleromyxedema is a rare, generalized form of lichen myxedematosus, which may be associated with systemic involvement and can be fatal. The therapeutic options available provide partial or inconsistent response and are associated with significant adverse effects. We report a case of scleromyxedema with cardiac involvement, treated with low-dose intravenous immunoglobulin, with almost complete c...

متن کامل

Managing scleromyxedema with intravenous immunoglobulin: acute worsening of scleromyxedema with biclonal gammopathy.

Scleromyxedema is a rare chronic cutaneous mucinosis usually associated with a monoclonal gammopathy and underlying systemic disease. The etiology of the disease is not known. There are no standard treatments and response to various therapeutic modalities varies. We report a case of refractory scleromyxedema in a 63-year-old man with a biclonal IgG and IgM λ-gammopathy. The patient was successf...

متن کامل

Acute Otitis Media-Induced Gradenigo Syndrome, a Dramatic Response to Intravenous Antibiotic

Introduction: Petrositis is a rare but severe complication of acute otitis media and mastoiditis. Despite efficient antibiotic therapy, there are still reports of both intratemporal and intracranial complications of otitis media with the potential risk of high morbidity and mortality. Petrositis has traditionally been treated with surgery, but recent advances in imaging, with improved antibioti...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Actas dermo-sifiliograficas

دوره 103 4  شماره 

صفحات  -

تاریخ انتشار 2012