Outland J David, Keiran Stephen J, Schikler Kenneth N, Callen Jeffrey P
Department of Medicine, Division of Dermatology, University of Louisville, Kentucky, USA.
Pediatr Dermatol. 2002 Nov-Dec;19(6):527-31. doi: 10.1046/j.1525-1470.2002.00226.x.
Multicentric reticulohistiocytosis is a rare multisystem disorder in which an infiltration of histiocytic cells causes papulonodular skin lesions and potentially a destructive polyarthritis. The active disease typically resolves spontaneously after 5-8 years, but the articular destruction can lead to permanent joint deformities. We present a case of multicentric reticulohistiocytosis in a 14-year-old girl. The number of papules on her hands decreased in number and her arthritic symptoms improved after 4 months of oral naproxen. Thirty months later her joint symptoms remained inactive and only six very small papules remained on her hands. Small flexor deformities were present in the distal interphalangeal joints of both index fingers. This case is an example of how multicentric reticulohistiocytosis can be a relatively stable and self-limited disease but still cause permanent joint deformities.
多中心性网状组织细胞增生症是一种罕见的多系统疾病，其中组织细胞浸润会导致丘疹结节性皮肤病变，并可能引发破坏性多关节炎。活动性疾病通常在5至8年后自发缓解，但关节破坏可导致永久性关节畸形。我们报告一例14岁女孩的多中心性网状组织细胞增生症。口服萘普生4个月后，她手上的丘疹数量减少，关节炎症状改善。30个月后，她的关节症状保持不活动状态，手上仅残留6个非常小的丘疹。双手示指远端指间关节存在轻度屈曲畸形。该病例表明，多中心性网状组织细胞增生症可以是一种相对稳定的自限性疾病，但仍可导致永久性关节畸形。
