Clin Rheumatol (2006) 25: 898–900
DOI 10.1007/s10067-005-0053-9
CASE REPORT
Gláucio R. Werner de Castro
.
Simone Appenzeller
.
Manoel B. Bértolo
.
Lílian T. L. Costallat
Acute dermatomyositis with subcutaneous generalized edema
Received: 30 April 2005 / Accepted: 6 May 2005 / Published online: 22 August 2006
# Clinical Rheumatology 2006
Abstract The authors report a 40-year-old Caucasian man
with relapsing muscle and skin involvement of dermato-
myositis treated with high-dose corticosteroids, taken
orally, and methotrexate and human gamma globulin,
both administered intravenously. After 4 months of ag-
gressive treatment, he presented with generalized edema,
considered secondary to dermatomyositis. Aggressive im-
munosuppression did not stop disease progression. The
literature concerning anasarca due to inflammatory my-
opathies is revised.
Keywords Dermatomyositis
.
Edema
.
Gamma globulin
Introduction
Polymyositis and dermatomyositis, the most common
causes of inflammatory muscle disease, are characterized
by inflammation of proximal striated muscle and typical
cutaneous lesions. Muscular involvement usually begins at
proximal limb muscles and, as disease progresses, may
affect esophagic and respiratory muscles [1].
Skin lesions, which characterize dermatomyositis, com-
prise the typical ones, heliotrope rash, Gottron’s papules,
and Gottron’s sign, aside from less characteristic lesions,
often erythematous and scaling, occurring at malar areas,
the neck, upper chest, and upper back. Cuticular over-
growth is also a frequent feature. Other relatively common
features are arthritis, calcinosis, interstitial lung disease,
and vasculitis [1].
Edema is not uncommon in eyelids and other sites with
loose, soft tissue, but generalized edema was rarely reported.
To the best of our knowledge, this is the 11th reported case
of generalized edema secondary to inflammatory myopa-
thies [2–8], and the fifth in patients with dermatomyositis [3,
5, 6, 8]. There are also some case reports of generalized
edema in juvenile dermatomyositis [9–11].
Diagnosis of dermatomyositis relies on typical clinical
manifestation, elevation of serum skeletal muscle enzyme
level, and characteristic electromyographic pattern, accord-
ing to Bohan and Peter [12] criteria.
Treatment begins with corticosteroids, but in most cases,
other drugs are necessary either to control the disease or to
allow reduction of corticosteroid dosage. Options include
methotrexate, azathioprine, cyclosporine, and human im-
munoglobulin. Chloroquine may be useful in management
of cutaneous lesions.
We reported a patient who had very active dermatomyositis
since its beginning, with respiratory muscle involvement,
poor response to intravenously administered corticosteroids
and methotrexate, and refractory skin involvement. After a
brief improvement with the use of immunoglobulin ad-
ministered intravenously, he presented progressive wors-
ening of muscle function and respiratory distress associated
with generalized edema, evolving to death despite aggres-
sive support.
Case report
A 40-year-old Caucasian man was referred to the Rheuma-
tology Unit of the State University of Campinas because of
progressive proximal muscular weakness of 4 months’
duration, heliotrope rash, and pruriginous, scaling, and ery-
thematous skin lesions in the trunk and limbs. He had been
taking low-dose prednisone during the previous month, but
his muscular weakness was gradually worsening. At the
time of the first evaluation in our service, he presented
proximal muscular strength of 2 of 5, tachypnea (respiratory
frequency of 30/min), tachycardia (pulse rate of 100/min),
and dysphasia. Chest X-ray evidenced aspiration pneumonia
treated with antimicrobials. The diagnosis of dermatomyo-
sitis was made based on the typical clinical picture, elevation
of serum muscular enzyme level (range 8,000–10,000;
G. R. Werner de Castro
.
S. Appenzeller (*)
.
M. B. Bértolo
.
L. T. L. Costallat
Rheumatology Unit, Department of Internal Medicine,
Faculty of Medical Sciences,
State University of Campinas (UNICAMP),
Distrito de Barão Geraldo,
13081-970 Campinas-SP, Brazil
e-mail: appenzel@unicamp.br