PMC5478574_01
The appearance and histological findings of the skin lesions. (A) The left panel shows multiple dark red erythemas of 3-4 mm in diameter with induration scattered on the trunk. The right panel shows a close view of the circled erythema, which was biopsied for a histological examination. (B and C) The examination of the biopsy specimen revealed inflammatory cell infiltration, consisting of lymphocytes and plasma cells, in the pereivascular areas and around the skin adnexa (Hematoxylin and Eosin staining). (D) Immunostaining showed an increase in the number of IgG4+ plasma cells with an IgG4+/IgG+ plasma cell ratio of 36%.
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On referral, multiple dark red erythemas of 3-4 mm in diameter with induration were scattered on the patient's face and trunk (Fig. 1A). A skin biopsy of an erythema on the patient's back revealed inflammatory cell infiltration consisting of lymphocytes and plasma cells in the perivascular areas and around skin adnexa (Fig. 1B and C). The IgG4+/IgG+ plasma cell ratio was 36% (Fig. 1D).
Magnetic resonance imaging (MRI) of the orbits, computed tomography (CT) of the chest, and the histology of the lacrimal gland. (A) Gadolinium-enhanced T1-weighted MRI shows the diffusely enhanced bilateral enlargement of the lacrimal glands (arrows) which had a slightly high-intensity on T2-weighted images and extended along the rectus muscles (triangles). (B) Chest CT reveals diffuse, faint, and centrilobular granular shadows, ground glass opacities with the thickening of the interlobular septa along the bronchovascular bundles, and multiple small air cysts with a thin wall (arrow). (C) A biopsy specimen from the lacrimal gland showed that the structure of the lacrimal gland was destroyed and replaced by dense lymphocyte and plasma cell infiltration (Hematoxylin and Eosin staining). (D) Immunostaining showed increased numbers of IgG4+ plasma cells and an IgG4+/IgG+ plasma cell ratio of 70%.
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Magnetic resonance imaging (MRI) revealed enlargement of the bilateral lacrimal glands, which extended along the rectus muscles (Fig. 2A). A biopsy of the enlarged lacrimal gland was performed, which revealed dense infiltration with lymphocytes and plasma cells, without atypia, and destruction of the lacrimal structure (Fig. 2C). The IgG4+/IgG+ plasma cell ratio was 70%; these findings were considered to be compatible with IgG4-RD (Fig. 2D). Chest CT showed the progression of the previously observed findings and the emergence of multiple small thin-walled air cysts (Fig. 2B).