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