Monika Bilewicz-Stebel, MD, Department of Internal Medicine, Dermatology and Allergology, Curie-Skłodowskiej 10, 41-800 Zabrze, Poland;
Acta Dermatovenerol Croat. 2020 Dec;28(3):190-192.
Eosinophilic fasciitis is a rare disease from the group of scleroderma-like connective tissue diseases with unclear etiopathogenesis. It may be occasionally accompanied with other eosinophilic or autoimmune dysfunctions (1,2). Lack of international diagnostic criteria and treatment consensus may lead to diagnostic and therapeutic difficulties. The 61-year-old man with no significant personal or family pathological history was admitted to the Dermatology Department presenting persistent induration for several months as well as erythema and pain of the shins that gradually extended to thighs and forearms, with limited mobility of peripheral joints. (Figure 1) Additional tests showed leukocytosis with 16% eosinophilia, elevated CRP, and hypergammaglobulinemia. Borrelia burgdorferi antibodies (classes IgM and IgG) were negative twice. A biopsy that included deep fascia was taken for histopathological examination. Antinuclear antibody screening was negative, but the direct immunofluorescence showed complexes in the dermo-epidermal junction and around the vessels. The diagnostics conducted toward malignant process showed no disturbing abnormalities (i.e. tumor markers in serum, chest, and abdomen computed tomography imaging, panendoscopy). The treatment was carried out with cephalosporin and nonsteroidal anti-inflammatory drugs (NSAIDs). The condition did not improve much but was stable. Histopathological results were indicative of eosinophilic fasciitis with fibrous thickening of deep fascia and perivascular infiltrations of plasma cells and lymphocytes; oral prednisone was initiated and the condition begin to improve. After 12 weeks, we observed disease progression with fever and very hard and cyanic skin lesions, which presented as an orange peel with linear furrows over the superficial venous vessels (Figure 2). The lesions extended to the trunk and caused troubles in moving. A complex rehabilitative intervention was started to minimize the inflammatory fascial restrictions. The prednisolone dose was increased, and oral methotrexate was added. After two weeks, the patient suffered from abdominal pain and periodic bleeding diarrhea. Methotrexate was suspected of inducing gastrointestinal adverse effects, and antipyretic NSAIDs were completely withdrawn. Colonoscopy showed features of mucosal edema with erythema, and histopathological examination revealed eosinophilic colitis. The patient was referred to a gastroenterologist, and methotrexate was ceased and switched to azathioprine. In summary, the consensus therapy of the rheumatologist, dermatologist, and gastroenterologist consisted of prednisolone and azathioprine. As of this writing, the patient's condition is gradually improving. The most characteristic symptoms of eosinophilic fasciitis is sudden onset with induration, sclerosis, and pain of the skin, with subcutaneous tissue and fascia usually appearing on the upper and lower limbs (3,4). The skin surface forms a characteristic orange peel appearance. The "groove" sign refers to the linear furrows over the superficial vessels of the extremities (1). Typical abnormalities are eosinophilia, elevated CRP, and hypergammaglobulinemia. The presence of eosinophilia is the most characteristic feature, occurring in 60-93% cases, but it is not necessary for diagnosis (1,5). Antinuclear antibodies are commonly absent with positive lesional direct immunofluorescence (6). If antinuclear antibodies are positive, it is recommended to broaden the diagnostic process to include other connective tissue diseases. Eosinophilia must be differentiated from hematological disorders and paraneoplastic syndrome. (4,6). Eosinophilic colitis is an eosinophilic gastrointestinal disease (EGID). It is the least frequent manifestation of EGID. It may be associated with connective tissue diseases, mostly systemic sclerosis - to our knowledge, there is no information in the literature about coexisting eosinophilic fasciitis. (7,8). The case described herein demonstrated that such a connection may occur. In treatment, it is important to prevent the patient from contractures and to maintain joint mobility by appropriate physiotherapy (2,9). The fascia forms a functional integral and continuous structure. Inflammation of one part of it changes the elasticity of the whole and produces fascial restrictions with movement limitation and pain. The fascia is profusely innervated, which favors constriction as a result of inflammation, and is also poorly vascularized which disrupts its regeneration (9,10). Myofascial techniques improve fascia elasticity by breaking up the tissue adhesions caused by inflammation (11). Eosinophilic fasciitis is a rare clinical entity, but knowing the possible clinical symptoms and laboratory abnormalities should help in taking the appropriate diagnostic path. It is important to treat the patient with attention to all concomitant diseases in consultation with different specialists.
嗜酸性筋膜炎是一种罕见的硬皮病样结缔组织疾病,其病因不明。它可能偶尔伴有其他嗜酸性或自身免疫功能障碍(1,2)。缺乏国际诊断标准和治疗共识可能导致诊断和治疗困难。这位 61 岁的男性无明显个人或家族病史,因小腿、大腿和前臂逐渐出现持续的硬结、红斑和疼痛,外周关节活动受限而被皮肤科收治。(图 1)其他检查显示白细胞增多,嗜酸性粒细胞 16%,C 反应蛋白升高,高丙种球蛋白血症。两次检测伯氏疏螺旋体抗体(IgM 和 IgG 类)均为阴性。进行了包括深筋膜的活检进行组织病理学检查。抗核抗体筛查阴性,但直接免疫荧光显示表皮-真皮交界处和血管周围有复合物。针对恶性过程的诊断检查未显示出任何异常(即血清肿瘤标志物、胸部和腹部计算机断层扫描成像、全内镜检查)。治疗采用头孢菌素和非甾体抗炎药(NSAIDs)。病情改善不大,但稳定。组织病理学结果提示嗜酸性筋膜炎,深筋膜纤维性增厚,伴浆细胞和淋巴细胞血管周围浸润;开始口服泼尼松,病情开始改善。12 周后,我们观察到疾病进展,出现发热和非常坚硬、发绀的皮肤病变,表现为浅静脉血管上呈橘皮样,有线性凹痕(图 2)。病变延伸到躯干,导致活动受限。开始进行复杂的康复干预,以最大限度地减少炎症性筋膜限制。增加泼尼松剂量,并加用口服甲氨蝶呤。两周后,患者出现腹痛和周期性腹泻。怀疑甲氨蝶呤引起胃肠道不良反应,完全停用解热 NSAIDs。结肠镜检查显示黏膜水肿伴红斑,组织病理学检查显示嗜酸性结肠炎。患者被转介给胃肠病学家,停用甲氨蝶呤,改用硫唑嘌呤。总之,风湿科、皮肤科和胃肠病学家的共识治疗包括泼尼松和硫唑嘌呤。截至本文撰写时,患者的病情正在逐渐改善。嗜酸性筋膜炎最典型的症状是突然出现皮肤硬结、硬化和疼痛,通常在上肢和下肢出现皮下组织和筋膜(3,4)。皮肤表面形成特征性的橘皮样外观。“沟”征是指四肢浅静脉上的线性凹痕(1)。典型异常包括嗜酸性粒细胞增多、C 反应蛋白升高和高丙种球蛋白血症。嗜酸性粒细胞增多是最具特征性的特征,发生在 60-93%的病例中,但不是诊断所必需的(1,5)。通常抗核抗体阴性,病变处直接免疫荧光阳性(6)。如果抗核抗体阳性,建议扩大诊断过程,包括其他结缔组织疾病。嗜酸性粒细胞增多必须与血液系统疾病和副肿瘤综合征相鉴别。(4,6)。嗜酸性粒细胞性结肠炎是一种嗜酸性粒细胞性胃肠病(EGID)。它是 EGID 中最不常见的表现。它可能与结缔组织疾病有关,主要是系统性硬化症-据我们所知,文献中没有关于同时存在嗜酸性筋膜炎的信息。(7,8)。本文描述的病例表明这种联系可能存在。在治疗中,重要的是通过适当的物理治疗预防患者出现挛缩并保持关节活动度(2,9)。筋膜形成一个功能完整且连续的结构。其一部分的炎症改变了整个筋膜的弹性,并产生筋膜限制,导致运动受限和疼痛。筋膜丰富地支配着,炎症导致其收缩,并且血管供应差,阻碍其再生(9,10)。肌筋膜技术通过打破炎症引起的组织粘连来改善筋膜弹性(11)。嗜酸性筋膜炎是一种罕见的临床实体,但了解可能的临床症状和实验室异常应有助于采取适当的诊断途径。重要的是要治疗患者,注意与不同专家咨询所有伴随疾病。