Dermatomyositis-like syndrome in x-linked agammaglobulinemia
Primary immunodeficiencies (PIDs) encompass more than 250 different pathological conditions. X-linked agammaglobulinemia (XLA) has been occasionally associated with cutaneous and muscular manifestations resembling dermatomyositis, often termed dermatomyositis-like syndrome (DLS). This syndrome has b...
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Sociedade Portuguesa de Reumatologia
2016-01-01
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doaj-4a7fd89816f34ff391952e443c0f70492020-11-24T22:36:08ZengSociedade Portuguesa de ReumatologiaActa Reumatológica Portuguesa0303-464X2016-01-01201617881CC140325Dermatomyositis-like syndrome in x-linked agammaglobulinemiaPedro David Carvalho0Carlos Costa1Marília Rodrigues2Maria João Salvador3José António Pereira da Silva4Armando Malcata5Centro Hospitalar e Universitário de CoimbraCentro Hospitalar e Universitário de CoimbraCentro Hospitalar e Universitário de CoimbraCentro Hospitalar e Universitário de CoimbraCentro Hospitalar e Universitário de CoimbraCentro Hospitalar e Universitário de CoimbraPrimary immunodeficiencies (PIDs) encompass more than 250 different pathological conditions. X-linked agammaglobulinemia (XLA) has been occasionally associated with cutaneous and muscular manifestations resembling dermatomyositis, often termed dermatomyositis-like syndrome (DLS). This syndrome has been associated with cutaneous, muscular and central nervous system manifestations, accompanying a persistent infection by an Echovirus. According to sixteen previously reported cases, this syndrome has a poor prognosis. We report the case of a 27-years old male, with XLA and DLS, successfully treated with 6 cycles of human immunoglobulin and methotrexate. Clinical symptoms improved dramatically with a complete resolution of the musculoskeletal manifestations. Despite this clinical response, prognosis should remain reserved. The evolution of this syndrome remains unpredictable and therapeutic options are limited. To the best of our knowledge, there are only a few reports of similar cases which have survived so many months after the diagnosis.http://www.actareumatologica.com/files/article/1091_dermatomyositis_like_syndrome_in_x_linked_agammaglobulinemia_file.pdfdermatomyositis-like syndromeX-linked agammaglobulinemiaImmunoglobulinprimary immunodeficiencies |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Pedro David Carvalho Carlos Costa Marília Rodrigues Maria João Salvador José António Pereira da Silva Armando Malcata |
spellingShingle |
Pedro David Carvalho Carlos Costa Marília Rodrigues Maria João Salvador José António Pereira da Silva Armando Malcata Dermatomyositis-like syndrome in x-linked agammaglobulinemia Acta Reumatológica Portuguesa dermatomyositis-like syndrome X-linked agammaglobulinemia Immunoglobulin primary immunodeficiencies |
author_facet |
Pedro David Carvalho Carlos Costa Marília Rodrigues Maria João Salvador José António Pereira da Silva Armando Malcata |
author_sort |
Pedro David Carvalho |
title |
Dermatomyositis-like syndrome in x-linked agammaglobulinemia |
title_short |
Dermatomyositis-like syndrome in x-linked agammaglobulinemia |
title_full |
Dermatomyositis-like syndrome in x-linked agammaglobulinemia |
title_fullStr |
Dermatomyositis-like syndrome in x-linked agammaglobulinemia |
title_full_unstemmed |
Dermatomyositis-like syndrome in x-linked agammaglobulinemia |
title_sort |
dermatomyositis-like syndrome in x-linked agammaglobulinemia |
publisher |
Sociedade Portuguesa de Reumatologia |
series |
Acta Reumatológica Portuguesa |
issn |
0303-464X |
publishDate |
2016-01-01 |
description |
Primary immunodeficiencies (PIDs) encompass more than 250 different pathological conditions. X-linked agammaglobulinemia (XLA) has been occasionally associated with cutaneous and muscular manifestations resembling dermatomyositis, often termed dermatomyositis-like syndrome (DLS). This syndrome has been associated with cutaneous, muscular and central nervous system manifestations, accompanying a persistent infection by an Echovirus. According to sixteen previously reported cases, this syndrome has a poor prognosis. We report the case of a 27-years old male, with XLA and DLS, successfully treated with 6 cycles of human immunoglobulin and methotrexate. Clinical symptoms improved dramatically with a complete resolution of the musculoskeletal manifestations. Despite this clinical response, prognosis should remain reserved. The evolution of this syndrome remains unpredictable and therapeutic options are limited. To the best of our knowledge, there are only a few reports of similar cases which have survived so many months after the diagnosis. |
topic |
dermatomyositis-like syndrome X-linked agammaglobulinemia Immunoglobulin primary immunodeficiencies |
url |
http://www.actareumatologica.com/files/article/1091_dermatomyositis_like_syndrome_in_x_linked_agammaglobulinemia_file.pdf |
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