单纯皮肤受累的成人朗格汉斯细胞组织细胞增生症一例

患者男,60岁, 躯干泛发褐色大斑片3年,斑片上及其周边出现结节半年合并溃疡2个月。患者3年前腹部皮肤出现红褐色斑片约1.0 cm × 2.0 cm,无明显自觉症状。当时未予任何处理,期间皮疹面积逐渐增大,并泛发到背部、臀部、侧腰部及腹股沟上方,颜色逐渐变深呈褐色,予口服及外用药治疗（具体不详）,皮损未见好转。半年前背部斑块上成簇出现粉红色黄豆大小结节,结节逐渐增大并变硬,数量增多,并很快发展到其他斑块及周围皮肤上,期间伴随着新结节的出现及老结节的消退……     

Sarcoidosis on skin scars: a case report

Sarcoidosis is a systemic inflammatory disorder of unknown origin, in which non-caseating granulomas (small inflammatory nodules) are found in the affected organs. Cutaneous involvement occurs in 25% of cases with a wide range of clinical presentation. The onset of scars is unusual although clinically characteristic of cutaneous sarcoidosis. Most patients with scar sarcoidosis have a systemic disease. It is reported the case of a 65 year-old woman that developed scar nodules 20 years after she had had surgical procedures without systemic manifestations. It is worth mentioning the importance of investigating sarcoidosis with inflammatory alterations in praevia scars.     

Multiple myeloma with skin involvement

Multiple myeloma (MM) is a plasma cell dyscrasia seen in the elderly that constitutes 10% of all haematopoietic neoplasias. It is a systemic disorder affecting various organs, in particular the kidneys and bones. Skin involvement is not a common finding. This report presents a case of MM with skin involvement.     

Goodpasture's syndrome associated with skin involvement

To our knowledge, skin lesions associated with Goodpasture's syndrome have not been reported to date. A patient developed discrete, macular, erythematous lesions on the instep of his foot, noted at the time of his admission to the hospital for treatment of Goodpasture's syndrome. Investigation showed perivasculitis and significant deposits of IgM and C3 at the dermoepidermal junction in the lesion site but not in the nearby skin. IgM deposits in the skin are unusual and the same immunoglobulin is found in the kidney in this syndrome. IgM deposit was absent from control samples of skin from four other patients with this syndrome. It is possible that these findings are significant.     

累及皮肤的套细胞淋巴瘤一例北大核心CSCD

患者女,51岁,确诊套细胞淋巴瘤20个月,四肢红色结节10 d.体检：左颈部及颌下区淋巴结肿大.双侧扁桃体Ⅱ度肿大.左上肢皮损组织病理示真皮及皮下组织见弥漫小淋巴细胞浸润,免疫组化示CD5、bcl-2、CD43、CD20、CyclinD1、λ均阳性,Ki-67阳性率＞70％,CD3、C10、bcl-6、CD23、CD21、κ均阴性.荧光原位杂交检测示t（11∶14）CCND1/IGH融合基因阴性.诊断：累及皮肤的套细胞淋巴瘤.治疗：给予环磷酰胺＋多柔比星＋长春新碱＋泼尼松方案治疗,病情控制,随访中.     

Cutaneous Sarcoidosis Clinically Mimicking Necrobiosis Lipoidica in a Patient with Systemic Sarcoidosis

A 70-year-old woman with an 8-year history of systemic sarcoidosis developed round, red-brown eruptions, with central atrophic lesions on her lower legs. The features of the biopsy specimen resembled those of necrobiosis lipoidica (NL), but although necrobiosis was present there were well-formed non-necrotizing granulomas in the dermis. The histological diagnosis was cutaneous sarcoidosis. Systemic sarcoidosis presenting with NL has rarely been reported. The histological features of cutaneous sarcoidosis sometimes mimic those of other granulomatous diseases, including NL and granuloma annulare, which are difficult to distinguish. We discuss the novel association between sarcoidosis and other granulomatous diseases.     

Necrotizing sarcoid granulomatosis with skin and pulmonary involvement

We present a rare case of necrotizing sarcoid granulomatosis (NSG) with skin and pulmonary involvement. NSG with extrapulmonary involvement occurs infrequently, and reports involving skin manifestations in NSG are even more rare.     

Granulomatous skin involvement in a patient with an unusual NOD2 mutation

Blau syndrome is a rare disorder that is classically characterised by granulomatous arthritis, skin eruptions and uveitis, which occur in the absence of lung involvement. Blau syndrome has been linked to encoding mutations in the NOD‐2 gene and is inherited in an autosomal dominant form. The most commonly observed mutations are missense substitutions affecting the arginine residue at position 334. The rare E600A mutation has been described as causing uveitis without skin involvement. Our patient is a 54‐year‐old man with an unusual heterozygous c.1799A>C(E600A) mutation, who was seen for bilateral lower extremity swelling and pain. On physical examination, he was found to have lower leg oedema with decreased hair growth on the affected area. Biopsy showed non‐caseating micro‐granulomas consistent with a diagnosis of Blau syndrome. The patient had excellent response to colchicine, but this was stopped because he developed elevated transaminases. Thus, we present an unusual genetic form of a rare condition and we demonstrate skin involvement in a subtype where cutaneous involvement has not hitherto been reported. In addition, the type and presentation of the skin involvement is different from that normally found in classic Blau syndrome. Finally, we report his response to colchicine, although it was ultimately not tolerated by this patient.     

单纯外阴受累的朗格汉斯细胞组织细胞增生症一例

患者女,44岁,因反复外阴溃疡8年,加重3个月于我科住院治疗。初起为外阴大量针头大小红色丘疹,伴痒,搔抓后破溃,形成糜烂面及溃疡,使用醋酸泼尼松片和复方甘草酸苷片、麝香等治疗后,外阴溃疡可暂时好转,但皮损反复。2013年因甲状腺结节行双侧甲状腺切除术……     

单纯外阴受累的朗格汉斯细胞组织细胞增生症一例

患儿女,5岁,因外阴丘疹、结节伴痒痛间歇发作3个月于2016年4月26日就诊。初发皮疹为阴唇多发的红色丘疹、结节,有痒痛感,曾于当地医院就诊,疑诊单纯疱疹病毒感染,给予抗病毒治疗及中药外用（具体不详）,无明显疗效。外阴皮损时轻时重,有时可自行缓解,之后再次发生,且逐渐加重,遂来山东大学齐鲁医院（青岛）就诊……