AJR 2002; 178:1233-1235
© American Roentgen Ray Society
Lupus Mastitis
Radiologic and Pathologic Features
Dianne Georgian-Smith1,2,
Thomas J. Lawton3,
Roger E. Moe4 and
William G. Couser5
1 Department of Radiology, Breast Imaging Section, University of Washington
Medical Center, 1959 N.E. Pacific St., Box 357115, Seattle, WA 98195.
2 Present address: Department of Radiology, Massachusetts General Hospital, Wang
ACC 219Q, 15 Parkman St., Boston, MA 02114.
3 Department of Pathology, University of Washington Medical Center, BB 210H,
Seattle, WA 98195.
4 Department of Surgery, University of Washington Medical Center, Box 356410,
Seattle, WA 98195.
5 Division of Nephrology, George M. O'Brien Kidney Research Center, University
of Washington Medical Center, Seattle, WA 98195.
Received June 7, 2001;
accepted after revision October 8, 2001.
Address correspondence to D. Georgian-Smith.
Introduction
This article describes imaging findings of a rare benign disease called
lupus mastitis, which is a subset of lupus panniculitis limited to the breast.
To our knowledge, the mammographic findings have not been documented in the
literature.
A 44-year-old woman with a remote history of lupus nephritis presented with
a breast mass. She developed a progressive disease, lupus mastitis, resulting
in a mastectomy. This disorder was a challenge to diagnose for all involved
physicians. A review of the clinical features and the progressive mammographic
findings are presented.
Case Report
A 44-year-old woman presented in 1998 with a painful left breast mass
associated with localized skin erythema. Her mammograms showed new
calcifications at the site of the palpable mass
(Fig. 1B). The findings from a
physical examination and mammography of the contralateral breast were normal.
Her medical history was significant for diffuse proliferative lupus nephritis
that was diagnosed in 1982. After 2 years of immunosuppression therapy, the
patient had stable renal function for more than 10 years. At the time of
presentation, she had no clinical or serologic evidence of systemic lupus
erythematosis and no history of trauma to the breast. The clinical diagnosis
before surgery was of a breast carcinoma with involvement of the skin.
However, the pathologic results from an excisional biopsy were clearly
negative for breast cancer but were confusing for lupus mastitis and lymphoma.
Histology showed necrotizing vasculitis and multiple areas of fat necrosis
with microcalcifications (Figs.
1D and
1E). Immunocytochemical studies
were performed that showed a mixed population of T and B lymphocytes and no
evidence of clonality, provisionally ruling out lymphoma.
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Fig. 1B. —44-year-old woman with lupus mastitis. Left breast mammogram
(mediolateral oblique view) obtained in February 1998 shows new segmental
heterogeneous calcifications (arrows). Some calcifications are
curvilinear and coarse, consistent with fat necrosis. Diffuse increased
density of fibroglandular tissues and decreased breast size are present.
Mammogram of contralateral breast (not shown) revealed normal findings and no
changes.
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Fig. 1D. —44-year-old woman with lupus mastitis. Photomicrographs of
histopathologic specimen of excised tissue show perivascular lymphocytic
infiltrate (H and E,x40) (D) and dense stromal fibrosis with
microcalcifications (H and E,x20) (E).
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Fig. 1E. —44-year-old woman with lupus mastitis. Photomicrographs of
histopathologic specimen of excised tissue show perivascular lymphocytic
infiltrate (H and E,x40) (D) and dense stromal fibrosis with
microcalcifications (H and E,x20) (E).
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Three months after the excisional biopsy, the symptoms worsened. The
patient presented with a painful nodular rash around the biopsy scar and a
breast mass in the surgical area. The mass was warm and tender and no
lymphadenopathy was present. Again, the provisional diagnosis of lupus
mastitis was made, although the patient had no symptoms of systemic lupus
erythematosis and the serology findings were negative. She was started on a
high dose of prednisone. Unfortunately, this regimen was poorly tolerated and
there was only partial compliance because of steroid-induced hallucinations.
The patient's symptoms improved in that the breast mass decreased, and she
experienced some relief of pain. Concurrently, however, her renal function
began to deteriorate.
By December 1999, the breast mass was enlarging and becoming extremely
painful, with associated skin erythema and ulceration. Clinically directed
core biopsies were performed to again rule out an advanced breast carcinoma
and lymphoma. The pathologic results of the core biopsies were similar to
those of the previous excisional biopsy. Two months later, the mass and the
ulcerated area had enlarged. The pain was interfering with the patient's sleep
and could not be controlled with steroids. Her renal function had deteriorated
so that she needed dialysis and was to be placed on the transplantation list
if lymphoma could be conclusively ruled out. As a last resort for relief of
her symptoms and to obtain a definitive histologic diagnosis, the patient
underwent a left breast mastectomy. For the third time, no carcinoma was
identified at pathology, and the diagnosis of lupus mastitis was
determined.
In the 18 months since the patient's mastectomy, she has had no further
symptoms of panniculitis in her chest wall and no symptoms of systemic lupus
erythematosis. She is doing well receiving dialysis and awaiting renal
transplantation.
Discussion
Kaposi [1] first proposed
the term "lupus panniculitis" in 1883. In 1940, Irgang
[2] introduced the term
"lupus erythematous profundus," for a disease in which involvement
of the breast is called lupus mastitis. Lupus mastitis is a rare chronic
inflammatory reaction of the subcutaneous fat that may occur in 2-3% of
patients with systemic lupus erythematosis
[3,
4], usually between the ages of
20 and 50 years, and its occurrence is two times greater in women than in men
[5]. Lesions most commonly
occur in the arms, buttocks, head, neck, and thighs. They are often firm, 3-cm
masses that may or may not be tender. As in this patient, epidermal changes of
atrophy and ulceration may be present
[4]. The clinical course of
lupus mastitis is often chronic with flares and remissions. Surgical excision
alone may not cure the patient if there is inadequate immunosuppression
[5,
6].
,
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Fig. 1C. —44-year-old woman with lupus mastitis. Left breast mammogram
(mediolateral oblique view) obtained in December 1999 shows diffuse coarse
dystrophic calcifications, increased density since 1998 of fibroglandular
tissues, and progressively smaller breast size. Architectural distortion
(arrows) is at site of prior biopsy. All imaging findings were
unilateral.
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The pathophysiology of lupus mastitis is unknown. One thought is that the
panniculitis is an extension of the inflammatory process that involves the
overlying skin. In cases without dermal involvement, the process may result
from vasculitis [4]. Another
theory proposes that lupus panniculutis can be exacerbated by localized
trauma, including biopsy [5],
but we do not think that there are enough cases to substantiate this belief.
No cases of lupus mastitis have been reported as preceding systemic lupus
erythematosis, and only rarely has lupus panniculitis been noted to occur
before systemic lupus erythematosis
[5].
This case of lupus mastitis occurred without clinical or serologic evidence
of systemic lupus erythematosis. Because of this unusual clinical setting,
there was a question as to the diagnosis of lupus mastitis. The diagnosis was
further confounded because of the paucity of data of the mammographic features
of lupus mastitis. Even though benign calcifications consistent with fat
necrosis were noted (Fig. 1B),
the cause of the heterogeneous calcifications was suspicious, particularly
because they were associated with a palpable mass and localized inflammatory
skin changes. Moreover, a literature search produced only one mammographic
image of lupus mastitis that, in retrospect, was more consistent with the
features that developed in our patient in 1999
[6], such as the large
confluent sheets of calcifications. Therefore, biopsies were recommended. It
is now clear that the heterogeneous calcifications were early stages of fat
necrosis that progressed, paralleling the clinical course so common to lupus
mastitis.
Lupus mastitis should not be confused with another rare disease, idiopathic
granulomatous mastitis [7]. The
clinical course of idiopathic granulomatous mastitis can also be one of
remissions and exacerbations. Dissenting opinions exist as to the benefits of
immunosuppression and surgical excision. The mammogram often shows normal
findings. Van Ongeval et al.
[7] presented MR imaging
findings of granulomatous mastitis in two lesions. One was an irregular
enhancing focus and the other was a ring-enhancing lesion thought to be a
granulomatous abscess. Histologically, granulomatous mastitis is a reaction
composed of lymphocytes, giant cells (histiocytes), and plasma cells with
occasional eosinophils. Sometimes the granulomatous reaction becomes confluent
with effacement of the lobular architecture and abscess formation. These
features do not occur in lupus mastitis. Moreover, granulomatous mastitis does
not have a lymphocytic vasculitis, as in our patient.
Because of the perivascular lymphocytic infiltrate in the breast tissues in
our patient, an untreated lymphoma such as breast cancer was also considered
to be causing the progressive symptoms. Lymphoma is most commonly a
metatstatic process of non-Hodgkin's lymphoma
[8]. Lymphoma often presents as
a palpable mass, sometimes with axillary adenopathy. Mammographic findings are
most commonly those of dense solitary masses and occasional multiple nodules.
The margins of the masses may be circumscribed to ill defined, but
desmoplastic reactions and calcifications have not been reported. An
infiltrative form presents with thickening of the skin
[8]. Therefore, lymphoma was
considered in the differential diagnosis. However, the skin ulceration and the
mammographic calcifications were not consistent with non-Hodgkin's lymphoma in
the breast. Immuno-cytochemistry was helpful in ruling out lymphoma in this
case.
In summary, this case of lupus mastitis was unusual in that no active
systemic lupus erythematosis was seen when the patient presented. The case
documents the progressive mammographic changes of lupus mastitis, including
the increasing density reflecting the development of fibrosis; the progression
in dystrophic calcifications from thin curvilinear calcifications to large
dense coarse ones; and the overall decrease in size of the breast, again
associated with the fibrosis. Awareness of the radiologic and clinical
features will be helpful in future diagnoses of lupus mastitis.
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Introduction
Case Report
