肉芽肿性皮肤松弛症

报告1例肉芽肿性皮肤松弛症.患者男,56岁.全身皮肤出现丘疹、结节25年,皮肤松弛10年.皮肤科检查:以褶皱部位为主全身可见300余个丘疹、结节,皮肤松弛下垂,未见隆起性及浸润性斑块.组织病理检查示真皮内以淋巴细胞为主的浸润,可见多核巨细胞肉芽肿,弹性纤维缺失.免疫组化染色示CD3、CD68、CD45RO均( ),CD20、CD79a均(-),Ki-67阳性细胞的比率<10%.     

肉芽肿性皮肤松弛症

肉芽肿性皮肤松弛症(granulomatous slack skin,简称GSS)是一种非常罕见的皮肤病^[1],属于T细胞淋巴瘤。临床上早期为红色或紫色斑块,表面萎缩,无自觉症状,好发于皮肤皱褶部位,如腋窝、腹股沟等,斑块逐渐增大,演变为巨大的皮肤包块,甚至悬垂在上述部位^[2]。组织病理具有特征性,表现为不典型T淋巴细胞浸润、多核巨细胞肉芽肿和弹力纤维缺失^[1]。该病发展缓慢,常伴发或先后出现其它的恶性淋巴增生性疾病。多数学者认为首例患者由Convit于1973年报道。van Haselen^[1]认为Bazex在1968年报道的病例可能是首例患 者。1978年Ackerman概括了该病的临床和病理特点,命名为“GSS”,并被接受沿用至今。迄今检索到的报道共37例。     

肉芽肿性皮肤松弛症1例

1 病例摘要患者女,32岁,右腋下褐红色斑10年,右腋下出现包块7年,无不适.患者10年前无明显诱因右腋下皮肤出现淡红色斑,表面干燥,少量脱屑,皮肤表面逐渐萎缩、起皱、松弛,无自觉症状,未作处理,后皮疹渐增大,累及右胸壁及肩部,7年前右腋下出现包块,局部无疼痛,无感觉缺失,多次求医未能确诊,曾诊断为血管瘤、淋巴瘤、组织细胞增生性肉芽肿等,用药具体不详,效果欠佳.患者自发病以来无发热,体重无明显减轻;发病前无用药史及创伤史;父母非近亲结婚,家族中无类似疾病患者,生有两子一女均健康,既往无特殊病史,在家务农,一直体健,无外出工作史.     

肉芽肿性皮肤松弛症1例

<正>1病例摘要患者女,32岁,右腋下褐红色斑10年,右腋下出现包块7年,无不适。患者10年前无明显诱因右腋下皮肤出现淡红色斑,表面干燥,少量脱屑,皮肤表面逐渐萎缩、起皱、松弛,无自觉症状,未作处理,后皮疹渐增大,累及右胸壁及肩部,7年前右腋下出现包块     

肉芽肿性皮肤松弛症一例

患者男，35岁，因皮肤红斑10个月于2000年10月来我院就诊。1年前患者上腹部有一皮下结节，直径约2cm，表面肤色基本正常，无自觉症状，在当地医院就医诊断为粉瘤，行手术切除，未做病理。术后2个月，手术部位逐渐红肿变硬，并向周围不断扩展。近2个月左胸部、近腋窝处又出现类似的小红斑而再次就诊。体检：脐上方2cm处有     

肉芽肿性皮肤松弛症4例分析

目的分析肉芽肿性皮肤松弛症(GSS)的临床病理特点、诊断和治疗,提高对本病的认识。方法回顾性分析近5年北京协和医院诊治的4例GSS患者的临床和实验室检查资料。结果 4例患者平均发病年龄25.5岁,早期临床表现不特异,最后均出现GSS典型的皮损及组织病理表现;患者有不同程度外周血嗜酸性粒细胞(EOS)计数、血清β2微球蛋白(β2-MG)和血清血管紧张素转换酶(ACE)水平升高,血清白蛋白(Alb)水平降低;3例皮损TCR基因重排阳性,1例阴性;予多种方法治疗后取得一定疗效。结论 GSS的诊断应结合临床特点、组织病理学、免疫组化、弹力纤维染色和基因重排检测。本病治疗困难,其标准治疗方案还需进一步研究。     

儿童肉芽肿性皮肤松弛症

肉芽肿性皮肤松弛症系一种罕见的T细胞性淋巴瘤,临床表现为皮肤松弛、斑块、结节。病理特点为皮肤的淋巴样细胞浸润及巨大的多核细胞性肉芽肿伴弹性纤维消失。本文报告1例男性患儿,10岁发病,15岁死亡。     

肉芽肿性皮肤松弛症

正肉芽肿性皮肤松弛症(granulomatous slack skin,GSS)是皮肤T细胞淋巴瘤中低度恶性的一型,临床罕见,现报道1例。临床资料患者,女,58岁。主因右臀部结节、斑块10余年,局部破溃疼痛3个月,于2014年5月22日就诊。患者10余年前无明显诱因右侧臀部反复出现大小不等的肤色或黄红色的丘疹、结节,偶有痒痛感。皮损数目逐渐增多,并有融合倾向。患者曾在多家医院就     

肉芽肿性松弛皮肤1例

右侧腹股沟下见直径约10cm的类圆形斑块,局部皮肤松弛图1肉芽肿性松弛皮肤临床照片1临床资料患者女,21岁。右侧腹股沟皮肤松弛并进行性加重6年。患者于6年前无明显诱因于右侧腹股沟处出现一指甲盖大小的红色斑片,向外逐渐增大,并稍隆起,皮损颜色逐渐呈紫红色,质软,呈袋囊状皮肤     

肉芽肿性皮肤松弛症六例临床及病理研究

目的 探讨6例肉芽肿性皮肤松弛症(GSS)的临床和病理特点.方法 收集6例GSS患者的临床和病理学资料.结果 6例均为男性,皮损为松弛斑块甚至垂悬状肿块,5例发生于皱褶部位(或为主),1例仅发生于右胸部.1例合并蕈样肉芽肿.组织病理均可见真皮内非干酪性肉芽肿,伴中等大小的淋巴样细胞、多核巨细胞浸润.1例见淋巴样细胞异形改变,余5例异形不明显.1例伴有亲表皮现象.免疫组化均见CD4+T细胞为主的单克隆增生.4例行弹性纤维染色见弹性纤维卷曲、断裂甚至消失,2例见多核巨细胞吞噬弹性纤维现象.2例行T细胞受体(TCR)基因重排,均为γ单克隆增生.2例患者采用浅层X线照射或电子束照射,皮损变小后手术切除,未再复发.其余4例患者采用干扰素α-2b肌内注射、0.02％氮芥外用等,症状有改善.结论 GSS进展缓慢,不推荐过度治疗.局限于皱褶部位的GSS推荐手术治疗.     

Granulomatous slack skin: successful treatment with recombinant interferon-gamma

Granulomatous slack skin is a rare variant of indolent cutaneous lymphoma, characterized by a cutis laxa-like clinical appearance and widespread granulomatous infiltration. A 25-year-old man had suffered from slowly progressive infiltrative, scaly and atrophic plaques and flaccid nodules with deep induration in the axillae, trunk and thighs. Histopathologically, dense lymphoid cell infiltration with numerous multinucleated giant cells (MGC) throughout the dermis to the subcutaneous tissue was observed. T-cell receptor gene rearrangement was detected in the skin lesions. Granulomatous infiltration with MGC was found in enlarged lymph nodes and the liver. Acute exacerbation was successfully treated with systemic recombinant interferon-gamma.     

Granulomatous slack skin

A 32‐year‐old man presented with multiple subcutaneous swellings of 6 years' duration. A biopsy performed 6 years earlier, when the patient presented with papules and plaques, was reported as tuberculoid Hansen's disease. The patient received dapsone, but showed no improvement. The disease was progressive. The patient currently has bilateral parotid enlargement ( Fig. 1 ), enlarged submandibular, submental, and axillary lymph nodes, multiple subcutaneous swellings over the axillae and arms of cricket ball size, loose skin folds in the axillae ( Fig. 2 ) and groin, and dry, scaly, and ichthyotic skin all over the body. Clinical differential diagnoses of mycosis fungoides and cutis laxa were made. The patient was discharged on request and then was lost to follow‐up.

Figure 1 Open in figure viewer PowerPoint Patient with bilateral parotid enlargement     

Granulomatous slack skin

A 39-year-old man had a two-year history of three indurated plaques that progressively underwent a cutis laxa-like transformation. Histopathologically a granulomatous, deep, dermal infiltrate was observed. It was composed mainly of numerous histiocytes and many multinucleate cells and foam cells. The case has the clinical and microscopic features of granulomatous slack skin (GSS), which may be considered to be a peculiar form of localized elastolysis. To date, there is no effective therapy for GSS.     

Granulomatous slack skin

Granulomatous slack skin is an extremely rare subtype of cutaneous T-cell lymphoma characterized by the slow development of folds of lax skin, especially in flexural areas and histologically characterized by a granulomatous infiltrate with clonal T cells. Notwithstanding its indolent behavior, treatment is often disappointing. We describe an additional case of this rare disorder in a 44-year-old male patient.