Case Report: Clinically amyopathic dermatomyositis presenting acutely with isolated facial edema [version 2; referees: 2 approved]
A 45-year-old Asian man presented with acute-onset periorbital and facial edema associated with pyrexia. Muscle weakness was absent. Initial laboratory investigations showed an inflammatory reaction, while screening for infections was negative. Serum muscle enzyme levels were normal. He was hospital...
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doaj-00991a1935714b0bb43b61edb2415cf62020-11-25T03:52:05ZengF1000 Research LtdF1000Research2046-14022018-03-01710.12688/f1000research.13604.215685Case Report: Clinically amyopathic dermatomyositis presenting acutely with isolated facial edema [version 2; referees: 2 approved]Efthymia Pappa0Marina Gkeka1Asimina Protogerou2Leonidas Marinos3Chariclia Loupa4Constantinos Christopoulos5Department of Internal Medicine, Amalia Fleming General Hospital, Athens, 15127, GreeceDepartment of Internal Medicine, Amalia Fleming General Hospital, Athens, 15127, GreeceDepartment of Internal Medicine, Amalia Fleming General Hospital, Athens, 15127, GreeceDepartment of Hematopathology, Evangelismos General Hospital, Athens, 10376, GreeceDepartment of Internal Medicine, Amalia Fleming General Hospital, Athens, 15127, GreeceDepartment of Internal Medicine, Amalia Fleming General Hospital, Athens, 15127, GreeceA 45-year-old Asian man presented with acute-onset periorbital and facial edema associated with pyrexia. Muscle weakness was absent. Initial laboratory investigations showed an inflammatory reaction, while screening for infections was negative. Serum muscle enzyme levels were normal. He was hospitalized and treated empirically with antibiotics and corticosteroids, pending the result of facial skin and muscle biopsy. He showed a good clinical and laboratory response but an attempt to discontinue corticosteroids led to a prompt relapse of facial edema and pyrexia, associated with rising laboratory indices of inflammation. Biopsy findings were typical of dermatomyositis. Reintroduction of corticosteroid treatment resulted in complete clinical and laboratory remission. Facial edema as the sole clinical manifestation of dermatomyositis is extremely rare. There have been no previous reports of isolated facial edema in the setting of acute, clinically amyopathic dermatomyositis in adults. A high level of suspicion is required to make the diagnosis in the absence of myopathy and the hallmark cutaneous manifestations of the disease (heliotrope rash, Gottron papules).https://f1000research.com/articles/7-60/v2 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Efthymia Pappa Marina Gkeka Asimina Protogerou Leonidas Marinos Chariclia Loupa Constantinos Christopoulos |
spellingShingle |
Efthymia Pappa Marina Gkeka Asimina Protogerou Leonidas Marinos Chariclia Loupa Constantinos Christopoulos Case Report: Clinically amyopathic dermatomyositis presenting acutely with isolated facial edema [version 2; referees: 2 approved] F1000Research |
author_facet |
Efthymia Pappa Marina Gkeka Asimina Protogerou Leonidas Marinos Chariclia Loupa Constantinos Christopoulos |
author_sort |
Efthymia Pappa |
title |
Case Report: Clinically amyopathic dermatomyositis presenting acutely with isolated facial edema [version 2; referees: 2 approved] |
title_short |
Case Report: Clinically amyopathic dermatomyositis presenting acutely with isolated facial edema [version 2; referees: 2 approved] |
title_full |
Case Report: Clinically amyopathic dermatomyositis presenting acutely with isolated facial edema [version 2; referees: 2 approved] |
title_fullStr |
Case Report: Clinically amyopathic dermatomyositis presenting acutely with isolated facial edema [version 2; referees: 2 approved] |
title_full_unstemmed |
Case Report: Clinically amyopathic dermatomyositis presenting acutely with isolated facial edema [version 2; referees: 2 approved] |
title_sort |
case report: clinically amyopathic dermatomyositis presenting acutely with isolated facial edema [version 2; referees: 2 approved] |
publisher |
F1000 Research Ltd |
series |
F1000Research |
issn |
2046-1402 |
publishDate |
2018-03-01 |
description |
A 45-year-old Asian man presented with acute-onset periorbital and facial edema associated with pyrexia. Muscle weakness was absent. Initial laboratory investigations showed an inflammatory reaction, while screening for infections was negative. Serum muscle enzyme levels were normal. He was hospitalized and treated empirically with antibiotics and corticosteroids, pending the result of facial skin and muscle biopsy. He showed a good clinical and laboratory response but an attempt to discontinue corticosteroids led to a prompt relapse of facial edema and pyrexia, associated with rising laboratory indices of inflammation. Biopsy findings were typical of dermatomyositis. Reintroduction of corticosteroid treatment resulted in complete clinical and laboratory remission. Facial edema as the sole clinical manifestation of dermatomyositis is extremely rare. There have been no previous reports of isolated facial edema in the setting of acute, clinically amyopathic dermatomyositis in adults. A high level of suspicion is required to make the diagnosis in the absence of myopathy and the hallmark cutaneous manifestations of the disease (heliotrope rash, Gottron papules). |
url |
https://f1000research.com/articles/7-60/v2 |
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