Lupus (2015) 0, 1–3

http://lup.sagepub.com

CASE REPORT

Lupus-like eruption as the presenting sign of acute

myeloblastic leukemia
F Cedeno-Laurent1, AE Obstfeld2, A Boni1 and JB Lipoff1
1
Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, USA; and 2Department of Molecular
Pathology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, USA

Cutaneous manifestations are often the presenting sign of internal malignancies. Up to 50% of
patients with acute myeloblastic leukemia (AML) have skin findings. Connective tissue dis-
ease, particularly dermatomyositis and, rarely, lupus, have been reported as a paraneoplastic
syndrome to various internal malignancies, but seldom with leukemias. We report the case of a
middle-aged woman ultimately diagnosed with acute myeloblastic leukemia, who presented
initially with a malar eruption, joint pains, and diffuse rash on the upper and lower extremi-
ties, with pathology consistent with lupus erythematosus and negative autoimmune serology.
There are no prior reports of cutaneous lupus as the presenting sign of AML, and this case
highlights the importance of considering paraneoplastic phenomena with presentations of
connective tissue disease in older patients, especially with negative serology. Lupus (2015)
0, 1–3.

Key words: Lupus; paraneoplastic; connective tissue disease; leukemia

Introduction trunk, and lower extremities, and was started on

prednisone 40 mg/day. Within days, the patient
Connective tissue disease as a paraneoplastic syn- became dyspneic and was admitted for work-up.
drome is extremely unusual for leukemias and Physical examination revealed a photodistributed
lymphomas, with no previous reports of lupus as erythematous eruption of her V-neck area, chin, and
the presenting sign of acute myeloblastic leukemia malar cheeks, with sparing of the nasolabial folds
(AML). (Figure 1(a)). The rash had a more violaceous com-
ponent by the eyelids. Multiple scattered, indurated
erythematous plaques were seen on her extensor
Case report arms and legs, and dorsal fingers, sparing the
joints (Figure 1(b), (c)). The patient reported mild
dyspnea, fatigue, and myalgias, and denied muscu-
Here, we report the case of a 52-year-old woman loskeletal weakness.
who presented with a four-week history of fatigue, Laboratory tests demonstrated pancytopenia with
diffuse myalgias/arthralgias, sweats, chills, and rash hemoglobin 8.1 g/dl, 117  103 platelets/dl and a
on her arms, interpreted as Lyme disease by her WBC 2  103 cells/dl (absolute neutrophil count
primary care physician. Her medical history was 340/dl), and sedimentation rate 101 mm/h (normal
remarkable for seronegative rheumatoid arthritis, <30 mm/h). Skin punch biopsies from representative
formerly treated with methotrexate, infliximab, lesions on her left arm and right and left thighs
and most recently, tocilizumab, but off therapy showed moderate perivascular, interstitial, and peri-
for the previous six months. After two weeks on adnexal lymphocytic infiltrates with increased inter-
doxycycline, she developed lesions on her face, stitial mucin, consistent with lupus or
dermatomyositis (Figure 1(d), (e)). Comprehensive
Correspondence to: Jules B Lipoff, Department of Dermatology, metabolic panel, B12/folic acid, C3/C4, TSH, creatin-
Perelman School of Medicine, University of Pennsylvania, Penn ine kinase and aldolase levels were all normal. In add-
Presbyterian Medical Center, Medical Arts Building, Suite 106, 51 N
39th Street, Philadelphia, PA 19104, USA.
ition, her anti-nuclear antibody, anti-Smith, anti-
Email: jules.lipoff@uphs.upenn.edu RNP, anti-Ro/La, anti-dsDNA, and myositis panel
Received 24 June 2015; accepted 16 September 2015 (anti-Jo-1, Mi-2, PL-7, PL-12, P155/140, EJ, Ku, U2
! The Author(s), 2015. Reprints and permissions: http://www.sagepub.co.uk/journalsPermissions.nav 10.1177/0961203315611497
 Lupus-like eruption as the presenting sign of acute myeloblastic leukemia
F Cedeno-Laurent et al.
2

Figure 1 Presenting cutaneous manifestation of newly diagnosed AML. Representative photographs of face (a), lower (b), and
upper extremities (c). Hematoxylin and eosin staining of lesional skin (left arm) showing interstitial and perivascular lymphocytic
infiltrate with prominent interstitial mucin (d). Periadnexal lymphocytic infiltration is depicted in (e).

sn, SRP, OJ, and PM/Scl) were all negative. Her
eruption rapidly cleared with prednisone 80 mg/day,
although her neutrophil count continued to drop.
Bone marrow biopsy revealed an expansion of a
small to medium sized mononuclear blast population
with irregular folded nuclei and inconspicuous
nucleoli, accounting for 30%–40% of cellularity,
with the immunophenotypical characteristics of
AML, with myelomonocytic features. The patient
initiated treatment with idarubicin and high dose
cytarabine, maintaining remission after consolida-
tion, and is awaiting bone marrow transplant.
Our patient had a clinical examination and
histopathology suggestive of lupus in the context
of initial presentation of AML. The malar ery-
thema with nasolabial sparing and the distribution
of plaques on the fingers, sparing the joints, was
most consistent with lupus, whereas the violaceous
tinge around her eyes also suggested dermatomyo-
sitis. Despite the physical examination and skin
Lupus
pathology supportive of connective tissue disease,
her serologic and other laboratory work-up was
negative, except for findings of AML.
Discussion
Lupus has only rarely been described as a paraneo-
plastic syndrome,1 and has not previously been
reported in association with AML. Still, hemato-
logic malignancies do occur in the lupus population
with greater frequency.2 Although dermatomyositis
is a well-established paraneoplastic syndrome, there
are only eight reported cases of dermatomyositis
with AML, and only two where both diagnoses
were made concurrently.3
In this case, amyopathic dermatomyositis cannot
be absolutely ruled out, but clinical features such as
sparing of the joints and nasolabial folds, in add-
ition to negative myositis screening for multiple

anti-synthetase antibodies, favor lupus. Lupus and
dermatomyositis have also been associated with
medications (hydralazine, procainamide, minocyc-
line, griseofulvin, hydralazine, and one case report
of doxycycline-induced lupus).3,4 Our patient
developed the malar erythema after receiving a
course of doxycycline. However, given that the ini-
tial eruption on the extremities preceded the initi-
ation of doxycycline, it seems unlikely that this
lupus-like eruption was caused by doxycycline-
induced photosensitivity. Further, the biopsy,
with findings consistent with connective tissue dis-
ease, was performed on the left arm from that ini-
tial eruption.
Rheumatoid arthritis significantly increases the
risk of developing AML, especially in those patients
treated with cyclophosphamide or azathioprine,5
though our patient had not received either.
Paraneoplastic presentations of connective tissue
disease are more likely to be serology negative
than idiopathic connective tissue disease presenta-
tions.5 Given this unusual presentation, we suggest
that clinicians should consider a paraneoplastic phe-
nomenon with any acute presentation of connective
tissue disease in adults over the age of 50, perhaps
even more so when serologies are negative.
Lupus-like eruption as the presenting sign of acute myeloblastic leukemia
F Cedeno-Laurent et al.
3
Declaration of Conflicting Interests
The authors declared no potential conflicts of inter-
est with respect to the research, authorship, and/or
publication of this article.
Funding
The authors received no financial support for the
research, authorship, and/or publication of this
article.
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Lupus