Good Response of Scleromyxedema and Dermato-Neuro Syndrome to Treatment With Intravenous Immunoglobulins☆

Resumen El escleromixedema es una enfermedad potencialmente grave que puede asociarse
a complicaciones sistemicas diversas, entre las que se encuentran las que involucran al sistema
nervioso central, siendo el sindrome dermato-neuro una de las mas infrecuentes. Se han
utilizado para su tratamiento farmacos variados, entre ellos, altas dosis de inmunoglobulinas
endovenosas. Se describen dos casos de escleromixedema, uno de los cuales desarrollo un sindrome
dermato-neuro, ambos con una excelente respuesta al tratamiento con inmunoglobulinas
endovenosas. El uso de altas dosis de inmunoglobulinas endovenosas en el tratamiento de esta
enfermedad y sus complicaciones neurologicas se perfila como un tratamiento de primera linea.
© 2011 Elsevier Espana, S.L. y AEDV. Todos los derechos reservados.

Escleromixedema y síndrome dermato-neuro: buena respuesta al tratamiento con glucocorticoides e inmunoglobulinas endovenosas: Good Response of Scleromyxedema and Dermato-Neuro Syndrome to Treatment With Intravenous Immunoglobulins

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 improvement of scleromyxedema with intravenous immunoglobulin. Herein we report a case showing dramatic improvement of scleromyxedema symptoms, both cutaneous and extracutaneous (including the dermatoneuro syndrome), and review the use of intravenous immunoglobulin in the treatment of scleromyxedema. This is a single case. The rarity of scleromyxedema, especially the dermatoneuro syndrome, precludes impedes large trials. In conclusion, increasing evidence supports intravenous immunoglobulin as an effective and relatively safe treatment for both cutaneous and extracutaneous manifestations of scleromyxedema, including the dermatoneuro syndrome.

Treatment of scleromyxedema and the dermatoneuro syndrome with intravenous immunoglobulin.

Papular mucinosis recurrent comas benign dysglobulinemia

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Papular mucinosis recurrent comas benign dysglobulinemia