PMC5684540_01
Skin lesions including palmar papules (a) and erythema on the back (b) before the initiation of treatment. A skin biopsy from the back indicates liquefactive degeneration with perivascular inflammation between the epidermis and dermis (c) (hematoxylin and eosin staining; scale bar = 100 microm).
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A physical examination demonstrated a body temperature of 37.7 C, mild muscular weakness of the proximal lower limbs, edematous hands, and cutaneous manifestations including a heliotrope rash, Gottron's papules, mechanic's hands, palmar papules, and an erythematous rash on his face and back; in particular, ulcerative and erosive erythema was visible on his elbows (Figures 1 and 2). The pathological finding of the biopsied skin from his back demonstrated perivascular infiltration of inflammatory cells with liquefactive degeneration through the epidermis to the dermis (Figure 1(c)).
Sequential findings of cutaneous lesions on the dorsum of the hand and elbow before the initiation of treatment (a, d), before the addition of rituximab (RTX) (b, e), and after RTX administration (c, f).
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A physical examination demonstrated a body temperature of 37.7 C, mild muscular weakness of the proximal lower limbs, edematous hands, and cutaneous manifestations including a heliotrope rash, Gottron's papules, mechanic's hands, palmar papules, and an erythematous rash on his face and back; in particular, ulcerative and erosive erythema was visible on his elbows (Figures 1 and 2). In addition, ulcerative and erosive eruptions on his hands and elbows also deteriorated (Figures 2(b) and 2(e)). Consequently, cutaneous involvements on his hands and elbows were remarkably improved (Figures 2(c) and 2(f)), and recovery from respiratory insufficiency and amelioration of the radiographic finding were also achieved (Figure 3(c)).
Chest computed tomography findings at admission (a), before the addition of rituximab (RTX) (b), and after RTX administration (c).
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Chest computed tomography (CT) revealed reticular shadows and ground-glass opacity on the middle to inferior fields of the bilateral lung (Figure 3(a)). Moreover, further exacerbation of the chest CT finding was obviously demonstrated (Figure 3(b)). Consequently, cutaneous involvements on his hands and elbows were remarkably improved (Figures 2(c) and 2(f)), and recovery from respiratory insufficiency and amelioration of the radiographic finding were also achieved (Figure 3(c)).
Clinical course of this patient. mPSL, methylprednisolone; PSL, prednisolone; IV-CsA, continuous intravenous infusion of cyclosporine A; oral-CsA, oral administration of cyclosporine A; IVCY, intravenous infusion of cyclophosphamide; RTX, rituximab.
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Since the patient was diagnosed with DM-related ILD and a low PaO2, methylprednisolone (mPSL) (1 g daily for 3 consecutive days) was immediately administered together with a continuous intravenous infusion of cyclosporine A (IV-CsA) according to the previously described therapeutic protocol (Figure 4). Furthermore, serum levels of ferritin and anti-MDA5-Ab decreased to 186 ng/mL and 44 indexes, respectively (Figure 4).