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- W2946245759 abstract "To the Editor: Granulomatous vasculitis in skin lesions is rarely seen in cutaneous sarcoidosis and other inflammatory skin disorders. We herein report a case of necrobiosis lipoidica, in which granulomatous phlebitis was observed in a muscular vein at the dermo–subcutaneous junction. A 59-year-old woman visited our hospital, complaining of asymptomatic eruptions of the lower extremities, which had appeared 3 years previously. She had been suffering from diabetes mellitus for several years and treated with oral hypoglycemic agents. Physical examination showed several well-circumscribed waxy brownish infiltrated plaques with elevated borders on the bilateral shins (Fig. 1A). Histological examination revealed necrobiotic changes of collagen in the dermis, surrounded by inflammatory granulomatous reaction composed of lymphocytes, histiocytes, and multinucleated giant cells (Figs. 1B, C). Furthermore, a muscular vein at the dermo–subcutaneous junction was surrounded by multinucleated giant cells in a style of a marked angiocentric infiltrate in and around the affected vessel wall with partial destruction of the muscular layer, which were consistent with the features of granulomatous phlebitis (Fig. 1D). Elastica van Gieson staining also confirmed that the involved vessel was a vein. Dermal mucin deposition was not detected. Immunohistochemistry results showed that the histiocytes in and around the affected vessel wall were immunoreactive for CD68 (Fig. 1E). On laboratory examination, the serum level of angiotensin-converting enzyme was normal, and neither PR3-ANCA nor MPO-ANCA was detected. Ophthalmological and pulmonary examination excluded sarcoidosis. The patient was treated with a topical difluprednate ointment, which, however, was not sufficiently effective.FIGURE 1.: A, Multiple waxy brownish plaques on the bilateral shins with slightly elevated erythema at the periphery. B, Histological features showing horizontally arranged palisading histiocytes and multinucleated giant cells surrounding degenerated collagen in the dermis (×40). C, Degenerated collagen surrounded by histiocytes and multinucleated giant cells (×40). D, Granulomatous phlebitis at the dermo–subcutaneous junction is characterized by a marked angiocentric infiltrate of histiocytes and multinucleated giant cells in and around the affected vessel wall with partial destruction of the muscular layer (×100). E, Intense CD68 staining also demonstrates a marked angiocentric infiltrate of histiocytes in and around the vessel wall (×200).Cutaneous disorders that histologically show granulomatous vasculitis include granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, rheumatoid arthritis, giant cell arteritis, Crohn disease, granulomatous phlebitis, and noninfectious granulomatous diseases such as sarcoidosis and necrobiosis lipoidica.1,2 In Ackerman's textbook,3 it is described that granulomatous vasculitis with histiocytes palisaded around vessels is observed in the deep dermis of necrobiosis lipoidica; however, there are very few reports of cases of necrobiosis lipoidica with granulomatous vasculitis. In ulcerative sarcoidosis, vasculitis is often suggested to be the cause. In a previous report of ulcerative necrobiosis lipoidica, giant cell chronic vasculitis was histologically revealed.4 By contrast, not all cases of ulcerative necrobiosis lipoidica show granulomatous vasculitis. Conversely, the present case did not present with ulcerative form, although granulomatous vasculitis was detected in the biopsied specimen. Phlebitis or thrombophlebitis in the lower legs is sometimes misdiagnosed as cutaneous arteritis because the veins in the lower legs have a compact concentric smooth muscle pattern with a round lumen and the intimal elastic fiber proliferation mimicking the characteristic features of arteritis.5 In the present case, the involved vessel was the muscular vein located at the dermo–subcutaneous junction. Magro et al6 suggested that the findings of active vasculopathy of leukocytoclastic, granulomatous, or thrombogenic subtypes in biopsied specimens of necrobiosis lipoidica or granuloma annulare should be considered as a manifestation of underlying systemic diseases. The pathogenesis of necrobiosis lipoidica is still uncertain; however, collagen degeneration, vascular changes, microangiopathy associated with diabetes, abnormalities in glucose transport by fibroblasts, impaired neutrophil migration, and inflammation triggered by altered metabolism or trauma have been speculated.7,8 Further studies regarding the association of granulomatous vasculitis with the clinical phenotypes of necrobiosis lipoidica are necessary." @default.
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- W2946245759 date "2020-04-01" @default.
- W2946245759 modified "2023-09-28" @default.
- W2946245759 title "Granulomatous Phlebitis in Necrobiosis Lipoidica" @default.
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- W2946245759 doi "https://doi.org/10.1097/dad.0000000000001458" @default.
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