Takahashi M, Mizutani H, Nakamura Y, Shimizu M
Department of Dermatology, Mie University, Faculty of Medicine, Tsu, Japan.
J Dermatol. 1997 Aug;24(8):530-4. doi: 10.1111/j.1346-8138.1997.tb02834.x.
We report the case of a 42-year-old Japanese woman who developed multicentric reticulohistiocytosis (MR) complicated by systemic sclerosis (SSc) and Sjögren syndrome (SS). The patient complained of tender nodules on the left hand, polyarthralgia in the finger joints and knees, and xerostomia. The skin nodules were distributed mainly on her hands and fingers with skin sclerosis. The serum anti-nuclear test revealed anti-centromere antibody and the discrete speckled pattern of anti-nuclear antibody. The biopsy specimens from the finger nodule and the sclerotic finger skin showed a perivascular infiltration of multinucleated giant cells with ground-glass cytoplasm and dermal thick collagen proliferation, respectively. The lip biopsy and sialography specimens showed periductal lymphocyte infiltration and apple tree-like changes. Systemic corticosteroid treatment improved the polyarthritis, xerostomia, and skin sclerosis rapidly but suppressed the nodular lesions only gradually. This is the first report of a combined case of MR, SSc and SS. This multiple autoimmune complication suggests the involvement of an immunological disturbance in the pathogenesis of MR.
我们报告了一例42岁的日本女性病例，该患者患有多中心网状组织细胞增多症（MR），并伴有系统性硬化症（SSc）和干燥综合征（SS）。患者主诉左手出现压痛性结节、手指关节和膝关节多关节疼痛以及口干。皮肤结节主要分布在双手和手指，并伴有皮肤硬化。血清抗核试验显示抗着丝点抗体以及抗核抗体的离散斑点模式。手指结节和硬化手指皮肤的活检标本分别显示多核巨细胞的血管周围浸润，其细胞质呈磨砂玻璃样，以及真皮胶原纤维增生增厚。唇部活检和唾液造影标本显示导管周围淋巴细胞浸润以及“苹果树样”改变。全身用皮质类固醇治疗迅速改善了多关节炎、口干和皮肤硬化，但仅逐渐抑制了结节性病变。这是首例MR、SSc和SS合并病例的报告。这种多种自身免疫并发症提示免疫紊乱参与了MR的发病机制。
