皮下结节病

皮下结节病是一种少见的结节病皮肤损害,病因不明,免疫反应是主要发病机制.临床表现为四肢的无痛性皮下结节,多伴有双侧肺门淋巴结肿大等系统损害.组织病理表现为皮下脂肪层和真皮深层的非干酪性上皮细胞样结节,周围有结缔组织包裹,结节周围少量淋巴细胞浸润.结合组织病理检查和影像学检查可明确诊断.糖皮质激素是首选治疗方法.肿瘤坏死因子-α拮抗剂、免疫抑制剂和光动力治疗也是可供选择的治疗手段之一.     

非感染性肉芽肿

20122059皮下结节病(综述)/周凌(广州军区武汉总医院皮肤科),晏洪波∥国际皮肤性病学杂志.-2012,38(3).-173～175结节病是一种多系统损害的非感染性肉芽肿性疾病,皮下结节型是其中相对少见的一种类型。皮下结节病的病因及发病机制尚不明了,遗传因素、微生物感染、化学物质和自身免疫等均可能是本病的病因之一。皮下结节病的临床表现主要为四肢的无痛性、可动性实性结节,单发或多发,多合并有双侧肺门淋巴结肿大等系统损害。组织病理表现为皮下脂肪层非干酪性上     

表现为红斑和皮下结节的结节病一例

报告1例同时表现为红斑和皮下结节的结节病。患者女,55岁。左眉暗红斑,颞部多发皮下结节2个月余。皮肤科检查:左侧眉弓直径约1 cm暗红斑,颞部多发皮下结节,直径0.5~1.5 cm,质硬,边缘不规则。皮损组织病理示:真皮或皮下脂肪层大量的上皮样细胞肉芽肿及多核巨细胞浸润,大部分呈裸结节。诊断:结节病。     

皮下结节型结节病

报告1例皮下结节型结节病.患者女,57岁.臀部及下肢红斑结节4个月伴疼痛1个月.皮肤科检查:臀部、双侧大腿红色结节,约蚕豆至核桃大,触之坚韧.皮损组织病理示:真皮中下部及皮下脂肪大量的上皮细胞肉芽肿及多核巨细胞,大部分呈裸结节.诊断为皮下结节型结节病.     

皮下型结节病一例并文献复习

患者,女,51岁。左膝肿块半年,右膝皮下结节1月余。双膝部皮损组织病理：真皮深部至皮下可见大的由组织细胞、上皮样细胞形成的结节,境界清楚,无干酪样坏死。网状纤维染色：（+）,结节周围有网状纤维增生。诊断：结节病（皮下型）。予羟氯喹联合复方甘草酸苷治疗1个月后,结节明显消退。     

系统性硬皮病并发皮肤结节病

患者女,54岁。发现四肢皮下结节1个月,系统性硬皮病病史20余年。皮肤科检查见"假面具脸",双手呈鹰爪样改变,四肢串珠状排列和散在分布大小不等的皮下结节,边界清楚,质硬,活动不明显,皮肤表观正常。皮损组织病理:皮下脂肪间隔多数结核样结构,大部分呈"裸结节",未见典型干酪样坏死,周围淋巴细胞很少。诊断:系统性硬皮病并发皮肤结节病。     

结节病5例临床分析

目的探讨皮肤结节病的临床特点,以减少误诊和误治。方法回顾性分析本科2009年7月-2011年5月诊治的5例皮肤结节病患者的临床表现、实验室检查、组织病理、误诊情况、治疗与转归等。结果 5例结节病患者皮损均累及面部,皮下结节型、红斑型、丘疹型、环状型和瘢痕型各1例,累及纵膈和肺门淋巴结较多,予强的松、复方甘草酸苷、白芍总苷胶囊等免疫调节治疗后病情好转。结论皮肤结节病皮疹临床表现各异,易被误诊,常侵犯肺、淋巴结和皮肤,引起系统损害,糖皮质激素治疗有效。     

非感染性肉芽肿

20140871皮下结节病一例／费良阅（北京中日友好医院皮肤科）,陈杨鑫,张晓艳∥实用皮肤病学杂志．-2013,6（5）．-303～304 患者女,52岁。四肢皮下结节反复发作4年余,有轻触痛,2～3月后皮损可自行消退,不留痕迹,反复发作。皮损组织病理：皮下脂肪中散在肉芽肿性结节,由上皮样细胞构成,无干酪样坏死,可见多核巨细胞,PAS染色（-）,抗酸染色（-）。诊断为皮下结节病,患者拒绝治疗。半年后随访,原有结节部分消退,未见遗留痕迹,有少量新疹,但前明显减轻,后失访。本病须与转移性Crohn病、皮肤结核、非典型分枝杆菌感染和深部真菌病鉴别。通常认为本病易伴发系统损害,但不发生骨损害、肺纤维化等严重系统病。预后相对较好。     

表现为获得性鱼鳞病的红皮病型结节病

报告1例以获得性鱼鳞病为首要表现的红皮病型结节病。患者女，27岁。双下肢片状鳞屑8个月。全身弥漫潮红、肿胀、脱屑，四肢、躯干散在较多皮下结节及肿块；腋下及腹股沟淋巴结增大；尿蛋白定量及肾功能均异常；心电图示心律异常；颈部B超示甲状腺非均质改变；CT示双腋窝淋巴结增大，纵隔内淋巴结增多，脾大；皮损组织病理检查和网状纤维染色确诊为结节病。经小剂量泼尼松治疗好转。     

伴骨损害的结节病

报告1例伴骨损害的结节病。患者女，37岁。下肢、面部、躯干皮疹10年余，右手示指、小指近端肿胀，活动受限3年。皮损组织病理检查示：真皮内可见由上皮样细胞构成的肉芽肿，中央无干酪样坏死，肉芽肿及其周围有淋巴细胞浸润。诊断：结节病。予甲泼尼龙、羟氯喹治疗后好转。     

Systemic sarcoidosis and cutaneous lymphoma: is the association fortuitous?

The association of systemic sarcoidosis and malignant lymphoma is known as the 'sarcoidosis-lymphoma syndrome'. Cutaneous involvement is rare in this syndrome. We report a 52-year-old woman who was diagnosed as having tumour-stage mycosis fungoides. Complete remission was achieved by combination therapy consisting of isotretinoin, interferon (IFN) alpha, electron beam irradiation, photochemotherapy and topical corticosteroids. Three years later, the patient developed systemic sarcoidosis characterized by yellowish papules on the abdominal wall and the eyelids that histologically revealed non-caseating granulomas, multiple fine-nodular interstitial pulmonary infiltrates on chest X-ray, hilar lymphadenopathy, decreased vital capacity and increased lymphocyte count in bronchoalveloar lavage fluid. As opposed to most of the reported cases, in our patient the manifestation of cutaneous lymphoma preceded the diagnosis of systemic sarcoidosis. We review the cases reported in the literature and discuss a possible causal and temporal relationship as well as the role of IFN alpha in the development of sarcoidosis.     

Sarcoidal granulomatous tenosynovitis of the hands occurring in an organ transplant patient

Six years after kidney-pancreas transplant, a 47-year-old white man developed multiple subcutaneous and tenosynovial nodules of hands and wrists, limiting mobility. Biopsy of multiple nodules showed fibrosing, sarcoidal granulomas, some of which contained pigmented material. Microbiology, immunohistochemistry, scanning electron microscopy with backscattered electron imaging and energy dispersive X-ray analysis and polymerase chain reaction assays failed to show any infectious agents or foreign material. There was no historical, clinical or laboratory evidence of systemic sarcoidosis. It is not known whether the donor had sarcoidosis. Despite empiric antimycobacterial therapy and ongoing immunosuppressive therapy (corticosteroids, mycophenolate, cyclosporine), the man has progressively developed more nodules, limiting hand function. Sarcoidosis occurring in non-donor tissue post-transplantation is an exceedingly rare complication of transplantation. We discuss this case and review the literature on sarcoidal tenosynovitis and sarcoidosis occurring post-transplantation.     

Relevant diagnostic implications of the therapeutic challenge with antitubercular therapy in an unusual case of sarcoidosis mimicking lupus vulgaris

Cutaneous manifestations in sarcoidosis are seen in 25–35% of patients with systemic disease and may be the sole manifestation in few patients. It is known that isolated cutaneous sarcoidosis is a great mimicker and can be easily misdiagnosed as other granulomatous conditions especially lupus vulgaris in regions with high burden of tuberculosis (TB). Here we present a case with cutaneous sarcoidosis who was initially misdiagnosed and treated as bifocal lupus vulgaris with antitubercular therapy (ATT) for 6 months. This nonresponsiveness to therapy prompted us to investigate the patient further for other differentials, failing which a diagnosis of cutaneous sarcoidosis was made and the patient was treated with oral steroids and methotrexate with complete clearance of lesions after 14 weeks of therapy. Our case reemphasizes the value of therapeutic trial of ATT in diagnosis of cutaneous TB and highlights the remarkable clinical mimic of sarcoidosis with lupus vulgaris.     

Photodynamic therapy as an alternative treatment for cutaneous sarcoidosis

Sarcoidosis is a systemic granulomatous disease. In more than 90% of the cases, sarcoidosis affects the lung with bilateral hilar adenopathy, while skin involvement occurs in about 25% of the cases. Whereas sarcoidosis of the lung is often successfully treated with oral corticosteroids, therapy of cutaneous sarcoidosis is frequently frustrating because some of the lesions may be refractory to treatment or recur quickly after resolution. We report two cases of cutaneous sarcoidosis successfully treated with photodynamic therapy, which represents an effective alternative therapy with fewer side effects.     

Skin uptake of Gallium 67 in cutaneous sarcoidosis

A patient is presented with cutaneous sarcoidosis of the scalp. Ga-67 was intensively taken up by the skin lesions. Systemic involvement of parotid glands and mediastinum was also demonstrated by Ga-67 scintigraphy. Prednisolone therapy reversed promptly the pathologic Ga-67 uptake. Ga-67 scintigraphy should be performed in all patients suffering from cutaneous sarcoidosis as being the most sensitive method to demonstrate systemic involvement.     

Sarcoidosis with extensive cutaneous ulceration. Unusual clinical presentation

A 70-year-old white woman with sarcoidosis and insulin-resistant diabetes mellitus presented with extensive cutaneous ulcerations. Both the cutaneous lesions and the systemic features of sarcoidosis showed a dramatic improvement during oral corticosteroid therapy. When extensive cutaneous ulcerations are present, it is important to consider sarcoidosis, as these may be the only presenting sign of the disease. Unlike ulcerated necrobiosis lipoidica diabeticorum, sarcoidal ulcerations may respond well to treatment with oral corticosteroids.     

Cutaneous sarcoidosis: an intriguing model of immune dysregulation

Sarcoidosis is a systemic granulomatous disease characterized by the presence of non‐caseating granulomas. Its etiology remains obscure. A plausible hypothesis suggests that a complex interplay of host factors, infectious processes, and non‐infectious environmental factors, matched with a susceptible genetic background, results in a pathway that leads to systemic granulomatous inflammation. Although presentations of sarcoidosis vary enormously, multi‐organ involvement is a common feature. Cutaneous involvement occurs in about 25% of patients with protean manifestations and variable prognoses. Skin manifestations are divided into specific lesions with histopathologically evident non‐caseating granulomas and nonspecific lesions arising from a reactive process that does not form granulomas. A peculiar form of cutaneous sarcoidosis is represented by sarcoidal lesions at sites of trauma that has caused scarring. The pathogenesis of scar sarcoidosis remains unknown. Scar sarcoidosis is also associated with herpes zoster infection, surgery, and tattooing. Such heterogeneous events, along with those at the sites of chronic lymphedema, thermal burns, radiation dermatitis, and vaccinations, occur on areas of vulnerable skin labeled “immunocompromised districts”. Numerous options are available for the treatment of cutaneous sarcoidosis. Although corticosteroids remain the treatment of choice for initial systemic therapy, other nonsteroidal agents have proven effective and therefore useful for long‐term management. Tumor necrosis factor‐α antagonists such as infliximab may have a role in the treatment of cutaneous sarcoidosis, especially in refractory cases that are resistant to standard regimens. Elucidation of the relationship of sarcoidal granulomas with malignancy and immunity may facilitate a better understanding of some pathomechanisms operating in neoplastic and immunity‐related disorders.     

Serum angiotensin-converting enzyme in sarcoidosis and psoriasis

Untreated pulmonary sarcoidosis is associated with an increased level of serum angiotensin-converting enzyme (SACE), which is regarded as a valuable method of diagnosing sarcoidosis and measuring the activity of the disease. The level of SACE in cutaneous sarcoidosis or other skin diseases has not been clearly established. We therefore examined SACE in 31 patients with systemic sarcoidosis, including cutaneous manifestations, and 12 patients with isolated cutaneous sarcoidosis. Also, 23 patients with psoriasis were studied. The level of SACE was generally elevated only in patients with untreated systemic sarcoidosis, whereas it was normal in cutaneous sarcoidosis and psoriasis. If the level of SACE is elevated in "isolated" cutaneous sarcoidosis, systemic disease must be strongly suspected.     

Acquired ichthyosis associated with an overlap syndrome of systemic sclerosis and systemic lupus erythematosus

Acquired ichthyosis is a condition accompanying many systemic illnesses such as lymphoma, sarcoidosis, dermatomyositis and systemic lupus erythematosus (SLE). Overlap syndromes are defined as clinical entities which satisfy each of the diagnostic criteria of two different connective tissue diseases concurrently or consecutively. The coexistence of SLE with systemic sclerosis has been very rarely reported. We describe a 33-year-old woman with an overlap syndrome consisting of systemic sclerosis and SLE who developed ichthyosis on her extremities.     

Sarcoidosis appearing in a tattoo

BACKGROUND: Sarcoidosis is a systemic disease that may present as tattoo granulomas. OBJECTIVE: A patient with systemic sarcoidosis who developed a granulomatous reaction within a tattoo is presented to stimulate interest in this unusual phenomenon. METHODS AND RESULTS: A patient with a 6-year history of pulmonary sarcoidosis developed sarcoidal granulomas restricted to one pigment of a tattoo. Previous reports of sarcoidal granulomas within tattoos are reviewed, and information about the pathogenesis of this process is explored. CONCLUSION: Sarcoid granulomas may develop in tattoos as an isolated local reaction or as the presenting sign of systemic sarcoidosis. The reaction itself may provide insight into further understanding the pathogenesis of sarcoidosis.