A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report.

The localized scleroderma (LS) known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. Thi...

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Autores:
Muñoz Buitrón, Evelyn
Bonilla Abadía, Fabio
Carrascal, Edwin
Cañas Dávila, Carlos Alberto
Ochoa, Carlos D.
Tipo de recurso:
Article of investigation
Fecha de publicación:
2012
Institución:
Universidad ICESI
Repositorio:
Repositorio ICESI
Idioma:
eng
OAI Identifier:
oai:repository.icesi.edu.co:10906/79015
Acceso en línea:
http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=3532086&tool=pmcentrez&rendertype=abstract
http://download.springer.com/static/pdf/387/art%253A10.1186%252F1756-0500-5-689.pdf?originUrl=http%3A%2F%2Fbmcresnotes.biomedcentral.com%2Farticle%2F10.1186%2F1756-0500-5-689&token2=exp=1478709616~acl=%2Fstatic%2Fpdf%2F387%2Fart%25253A10.1186%25252F1756-0500-5-689.pdf*~hmac=d3be4790122e9ed1059408192b18559a9d5c60e7b0151803736e91f2932169ac
http://hdl.handle.net/10906/79015
http://dx.doi.org/10.1186/1756-0500-5-689
Palabra clave:
Economía
Econometría
Economics
Econometrics models
Vasculitis
Sistema nervioso central
Enfermedades sistémicas
Enfermedades autoinmunes
Rights
openAccess
License
https://creativecommons.org/licenses/by-nc-nd/4.0/
Description
Summary:The localized scleroderma (LS) known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our knowledge this is the first case of a morphea forming part of a multiple autoimmune syndrome (MAS) and presenting simultaneously with autoimmune thrombocytopenic purpura and central nervous system vasculitis. CASE PRESENTATION: We report an uncommon case of a white 53 year old female patient with LS as part of a multiple autoimmune syndrome associated with pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis presenting a favorable response with thrombopoietin receptor agonists, pulses of methylprednisolone and cyclophosphamide. CONCLUSION: Is likely that LS have an autoimmune origin and in this case becomes part of MAS, which consist on the presence of three or more well-defined autoimmune diseases in a single patient.