Yamamoto Toshiyuki
From the Department of Dermatology, Fukushima Medical University, Fukushima, Japan.
J Clin Rheumatol. 2022 Jan 1;28(1):e234-e239. doi: 10.1097/RHU.0000000000001679.
Multicentric reticulohistiocytosis (MRH) is a rare multisystem disorder, primarily affecting the skin and joints. As severe joint damage is a possible symptom, early diagnosis and therapeutic intervention are required. Cutaneous lesions present with characteristic features such as discrete reddish nodules, especially on acral locations. Additionally, the face, scalp, trunk and extremities are also affected. Xanthomatous plaques are also seen on the face. The cutaneous manifestations of MRH are various, which therefore should be differentiated from several diseases. In particular, MRH has been reported to assume clinical appearances resembling Gottron papules, periungual erythema, V-neck erythema, shawl sign, and poikiloderma associated with dermatomyositis. Histopathologic features show proliferation of multinucleated giant cells with abundant eosinophilic granular ground glass-like cytoplasm in the dermis. Multicentric reticulohistiocytosis is occasionally paraneoplastic and is associated with internal malignancies. The appropriate therapies are still challenging, and oral prednisolone, immunosuppressants, and recent biologics are used. In this article, cutaneous lesions, histopathology, and pathogenesis of MRH are mainly discussed from a dermatological perspective. It is important, not only for dermatologists but also for rheumatologists and orthopedists, to be able to distinguish between the various types of skin lesions brought on by MRH. Cutaneous signs are important diagnostic clues and assessment tools for therapeutic efficacy.
多中心网状组织细胞增生症（MRH）是一种罕见的多系统疾病，主要累及皮肤和关节。由于严重的关节损伤是一种可能出现的症状，因此需要早期诊断和治疗干预。皮肤病变具有特征性表现，如散在的红色结节，尤其是在肢端部位。此外，面部、头皮、躯干和四肢也会受累。面部还可见黄色瘤样斑块。MRH的皮肤表现多种多样，因此应与多种疾病相鉴别。特别是，据报道MRH可呈现出类似于皮肌炎的Gottron丘疹、甲周红斑、V领红斑、披肩征和皮肤异色症的临床表现。组织病理学特征显示真皮内多核巨细胞增生，伴有丰富的嗜酸性颗粒状玻璃样细胞质。多中心网状组织细胞增生症偶尔为副肿瘤性，与体内恶性肿瘤有关。合适的治疗方法仍然具有挑战性，目前使用口服泼尼松龙、免疫抑制剂和近期的生物制剂。在本文中，主要从皮肤病学角度讨论了MRH的皮肤病变、组织病理学和发病机制。对于皮肤科医生以及风湿病学家和骨科医生来说，能够区分由MRH引起的各种类型的皮肤病变非常重要。皮肤体征是重要的诊断线索和治疗效果评估工具。
