Acta Dermato-Venereologica 99-1CompleteContent | Page 23
SHORT COMMUNICATION
95
Acquired Partial Lipodystrophy Associated with Anti-Mi-2 Antibody-positive Adult-onset
Dermatomyositis
Ken SHIRAISHI, Mikiko TOHYAMA and Koji SAYAMA
Department of Dermatology, Ehime University Graduate School of Medicine, 791-0295 Toon, Ehime, Japan. E-mail: [email protected]
Accepted Aug 31, 2018; Epub ahead of print Sep 3, 2018
Acquired partial lipodystrophy (APL) is a rare disorder
characterized by the loss of subcutaneous adipose tissue,
usually starting in the face and extending to the neck,
arms, and trunk (1). The exact pathogenesis remains
unclear, but it can be associated with metabolic disorders
and autoimmune diseases, particularly juvenile dermato-
myositis (JDM) (2). Here we report a rare case of APL in
an adult patient with anti-Mi-2 antibody positive derma-
tomyositis who did not develop metabolic abnormalities.
CASE REPORT
A 53-year-old woman presented to our institution with a 3-month
history of erythematous eruptions on the face, upper trunk, extre-
mities, and dorsal surfaces of both hands. Physical examination
revealed diffuse edematous erythema on the back and thighs
(Fig. 1A, B). We also detected erythematous lesions on the dorsal
surfaces of both hands, periorbital erythema and Gottron papules
(Fig. 1C), but no periungual telangiectasia was evident. The pa-
tient showed no evidence of myositis. Blood testing showed that
complete blood count, liver and renal function test and urinalysis
were all normal. The results of antinuclear antibody testing were
positive, with a titer of 1:2560. C3 and C4 fractions of the comple-
ment were within normal limits. C3 nephritic factor (C3NeF) was
not available in our hospital. Anti-ARS, anti-MDA5, anti-TIF-1γ,
anti-RNP, anti-Sm, anti-ssDNA, and anti-dsDNA antibody tests all
yielded negative results. Positive results were obtained for anti-
Mi-2 antibody (> 150). No abnormalities in serum lipid or glucose
levels were detected. Computed tomography demonstrated mild
interstitial pneumonia.
Skin histopathology from erythematous lesions showed interface
dermatitis with perivascular infiltration of lymphocytes in the
dermis (Fig. 1D), consistent with dermatomyositis. Cancer screens
revealed no indication of malignancy and ruled out underlying neo
plastic disease. Since the patient showed no evidence of systemic
disorder, organ damage and myositis, we followed-up skin lesions
by topical steroid ointment. Skin lesions then started to gradually
improve. However, after a 1-year follow-up period, she mentioned
a progressive loss of fat in the facial region over the last several
months, after her family pointed out changes in her face. She had
no history of dieting, and had never used drugs associated with
lipodystrophy. No family members had any history of connective
tissue disease or lipodystrophy. Physical examination revealed a
marked loss of adipose tissue in the face, particularly as bilateral
loss of the buccal fat pads and prominence of the zygomatic arches
and forehead (Fig. 2D–F). Loss of some fat on both proximal arms
were also seen. The trunk and lower limbs appeared unaffected.
Based on the above findings, the diagnosis of APL was made.
DISCUSSION
Fig. 1. Diffuse edematous erythema on the back (A) and thighs (B).
C) Erythematous lesions on the dorsal surfaces of both hands. Periorbital
erythema and Gottron papules were also seen. D) Histopathological findings
of biopsy tissues show interface dermatitis with vacuolar change with sparse
superficial perivascular infiltration of lymphocytes. Hematoxylin-eosin stain,
original magnification: ×100.
Lipodystrophy is a group of adipose tissue disorders
classified into genetic/acquired or generalized/partial
lack of adipose tissue (lipoatrophy) (1). APL presents
with late-onset symmetrical loss of fat from the face,
neck, arms, and trunk. Females are affected four-times
more often than males. In our case, the female patient
showed severe loss of adipose tissue in the face and
upper extremities with sparing of the lower extremities,
consistent with APL.
APL has been associated with infections, antiretroviral
therapy for the treatment of human immunodeficiency
virus infection and autoimmune diseases such as rheu-
matoid arthritis, systemic lupus erythematosus, Sjögren
syndrome, and dermatomyositis (3). Dermatomyositis is
the autoimmune disease most frequently related to the
development of lipodystrophy (4), but most cases are
specifically associated with JDM. JDM is characterized
by constitutional symptoms, pathological calcifications,
skin infarctions, lipodystrophy, and a lack of internal
malignancy (5). The prevalence rates of lipodystrophy
in JDM vary from 10% to 40% (2), but only 4 cases of
lipodystrophy affecting adult-onset dermatomyositis
patients have been reported to date (6–9). To the best
of our knowledge, APL associated with adult-onset
This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta
Journal Compilation © 2019 Acta Dermato-Venereologica.
doi: 10.2340/00015555-3026
Acta Derm Venereol 2019; 99: 95–96