ARTICLE
The rash in dermatomyositis is often diagnostic; a violaceous heliotrope
erythema occurs on the face, especially involving the eyelids, upper cheeks,
forehead and temples. Small, erythematous or violaceous papules occur
on the dorsum of finger joints, elbows or knees [1]. Flagellate erythema
on the trunk and proximal extremities is also observed in dermatomyositis
[2-7]. Dermatomyositis in adult patients is sometimes associated with
an internal malignancy [8] and worsens with the progress of the neoplasm
[1]. The rash sometimes occurs without muscle involvement, which is referred
to as amyopathic dermatomyositis [9, 10]. We report here amyopathic dermatomyositis
associated with breast cancer in a patient who had skin metastasis on
the back with a clinically similar appearance to amyopathic dermatomyositis.
Case report
A 65-year-old Japanese woman suffering from wide spread pruritic erythema
on the back for 2 months presented at our department on May 25, 1998.
Partially flagellate erythema was found on the back (Fig.
1A) and upper extremities, but not on the face. Telangiectasia
and livedo lesions were seen on the lumbar region. Small erythematous
papules were observed on the dorsum of the PIP, DIP and MP joints and
erythema around the nail folds. Histopathological findings of the biopsy
specimen from erythema on the back showed mononuclear cell infiltration
around capillaries and marked edema in the upper dermis (Fig.
2A). The deposition of IgG, IgA, IgM, C3, and C1q were all negative
by direct immunofluorescence study. She had undergone total left mastectomy
on June 2, 1997 because of breast cancer and left axillary lymph node
metastasis. She noticed supraclavicular lymph node swelling at the beginning
of May and the metastasis was certified histologically on May 29, 1998.
Laboratory data were within normal range including serum creatine phosphokinase
(CPK), aldolase and myoglobin, but she was positive for anti-nuclear antibody
(x 80 speckled type). Anti-Jo-1 antibody was negative. She did not report
muscle weakness, gait disturbance or general fatigue. Muscle biopsy was
not performed because the patient did not consent. A diagnosis of amyopathic
dermatomyositis associated with breast cancer was made. She was treated
with oral administration of oxatomide 60 mg/day and hydroxyzine 25 mg/day
for 6 weeks, followed by epinastine hydrochloride 10 mg/day for 6 weeks
and topical betamethasone valerate ointment, but skin eruptions and itching
continued. Radiation to the neck lymph nodes (50 Gray) and chemotherapy
of 4 courses of CMF (100 mg/day cyclophosphamide for 14 days, 60 mg/day
methotrexate for 2 days and 500 mg/day fluorouracil for 2 days) were given
from June 23 to October 3, 1998. These treatments reduced the swelling
of the supraclavicular lymph nodes but complete remission was not obtained.
Erythema with itching gradually subsided from the end of August and disappeared
by the end of October.
She received treatment of oral administration
of tamoxifen citrate 20 mg/day from July 7 1997 and 600 mg/day doxifluridine
was added from December 18, 1998. Toremifene citrate (40 mg/day) was given
from December 13, 1999 instead of tamoxifen. The sizes of the supraclavicular
lymph nodes were increased from the beginning of May and lung metastasis
was detected on chest X-ray, chest computed tomography and biopsy in the
middle of June, 1999. Therefore, 80 mg docetaxel hydrate was given every
4 weeks for 8 months from May 1999 to January 2000, but the sizes of the
supraclavicular nodules increased. Erythema without itching re-appeared
on the back but not on the breast from the beginning of January, 2000
(Fig. 1B). Laboratory
data were within normal range including CPK, aldolase and myoglobin except
for positive anti-nuclear antibody. She did not report muscle weakness,
gait disturbance or general fatigue. Histological findings of skin biopsy
specimens from the back showed that many carcinoma cells positively stained
by PAS stain, which were similar to the primary lesion of left breast
cancer, were scattered in the whole dermis (Figs.
2B and 2C). Mononuclear
cell infiltration around capillaries and slight edema were also seen in
the dermis. A diagnosis of skin metastasis of breast cancer was made at
that time. She complained of dyspnea due to pleural effusion in the middle
of February and died of respiratory failure on March 13, 2000 (Fig.
3).
Discussion
In this case, a diagnosis of amyopathic dermatomyositis associated with
breast cancer was made from clinical signs and histological findings in
May, 1998. The patient had undergone mastectomy in June, 1997 for breast
cancer, but supraclavicular lymph node metastasis was found in May, 1998.
The skin lesions improved following a reduction in the sizes of lymph
nodes by radiation and chemotherapy. However, lung metastasis was found
in June, 1999. Erythema on the back appeared again in January, 2000, showing
histologically many carcinoma cells scattered in the whole dermis. A diagnosis
of skin metastasis was made at that time. The erythema on the back in
2000 was clinically similar to that seen in 1998, but there were some
differences. Initially, itching and involvement of the upper extremities
and the dorsum of finger joints were observed in the erythema in 1998.
Additionally, the skin eruptions in 1998 were flagellate erythema. Although
linear erythematous lesions are generally suggestive of cutaneous processes
affected by external stimuli such as contact or factitious causes, the
characteristic flagellate erythema are also seen in cases with the use
of bleomycin or in dermatomyositis [7]. Flagellate erythema is very characteristic
of dermatomyositis and has not been associated with other connective tissue
disorders [6]. Thus, the presence of flagellate erythema is a useful clinical
sign for the diagnosis of dermatomyositis and for distinguishing dermatomyositis
from other cutaneous diseases.
Metastatic skin lesions of breast cancer most
commonly show cutaneous or subcutaneous nodules with erythema ranging
from tiny 1-2 mm lesions to large masses. The nodules are usually asymptomatic,
but sometimes painful [11]. Metastatic breast cancer can also present
as an erysipelas-like eruption. Although erythema without a nodule as
shown here is not common in metastatic breast cancer, skin metastasis
should be taken into account for patients with erythema on the trunk clinically
similar to dermatomyositis. Since the erythema which appeared again on
the back in 2000 showed histologically many carcinoma cells scattered
in the whole dermis, the diagnosis of skin metastasis of breast cancer
was made. However, mononuclear cell infiltration in the dermis was observed,
suggesting that it might be also involved in erythema formation.
Three patients with breast cancer who responded completely or partially
to tamoxifen and developed dermatomyositis within three months of tumor
regression have been reported [12]. Although the causes of dermatomyositis
are still unknown, a possible involvement of tamoxifen therapy itself
or tumor regression by this agent in the onset of dermatomyositis is raised
[12]. Thus, the possibility that tamoxifen may have been related to the
onset of amyopathic dermatomyositis in this patient cannot be neglected.
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