淋巴细胞性原发性瘢痕性脱发2例

报告2例淋巴细胞性原发性瘢痕性脱发。例1.患者男,53岁。头皮多发红斑及脱发1年。皮肤科检查:头部多发椭圆形花生米大脱发斑,局部头皮萎缩。皮损组织病理检查:表皮角化过度,毛囊角栓,棘层灶性变薄,毛囊上皮基底细胞液化,真皮浅层血管附属器周围大量淋巴细胞浸润。直接免疫荧光(DIF)示真、表皮交界处,真皮与毛囊上皮交界处线状及颗粒状C3(+)、IgM(±)、IgG(-)及Ig A(-)。免疫组化:抗核抗体(ANA)(+)1∶40、抗SSA抗体(++)及抗线粒体抗体(+)。例2.患者女,48岁。头皮多发红斑伴脱发和瘙痒2年。皮肤科检查:头皮多发形状不规则的脱发斑片,部分融合,局部头皮萎缩。皮损组织病理检查:表皮角化过度,毛囊角栓,毛囊上皮基底细胞液化,附属器周围大量炎性细胞浸润。DIF(-)。免疫组化:ANA(-)。2例患者均诊断为淋巴细胞性瘢痕性脱发。     

脂水肿性脱发1例

患者女,62岁,头皮肿胀且局部横沟伴压痛半年。皮损组织病理示:表皮大致正常,真皮浅中部血管周围少量淋巴单核细胞浸润,胶原间纤维母细胞及胶原增生,真皮中下部毛囊周围见成熟脂肪细胞增生;黏蛋白染色示:毛囊周围及毛乳头见少量黏蛋白沉积。综合临床和病理表现,该患者诊断为脂水肿性脱发,脂水肿性头皮。     

小剂量利妥昔单抗联合糖皮质激素治疗儿童落叶型天疱疮一例疗效观察

患儿男,9岁,头皮红斑、糜烂伴瘙痒1个月,泛发全身1周。皮损组织病理：表皮内颗粒层下方水疱形成,疱内见大量棘层松解细胞,真皮浅层小血管周围淋巴细胞及嗜酸性粒细胞浸润。直接免疫荧光检查示表皮细胞间IgG、补体C3呈网状沉积,IgM、IgA阴性。酶联免疫吸附实验检测血清抗Dsg1抗体阳性（157.00 U/ml）。诊断：落...     

毛囊黏蛋白病

报告1例毛囊黏蛋白病。患者男,33岁。全身泛发红斑伴瘙痒13年余,加重近半年,并出现眉毛脱落。皮损组织病理检查示：真皮毛囊周围炎性细胞浸润。阿新蓝染色阳性,毛囊内可见黏蛋白沉积。诊断为毛囊黏蛋白病。     

不伴粘蛋白沉积的嗜毛囊性蕈样肉芽肿1例

报告不伴粘蛋白样沉积的嗜毛囊性蕈样肉芽肿1例。患者男,39岁。全身浅表淋巴结肿大10年,全身红斑、毛发脱落2年。组织病理示:淋巴细胞侵入表皮内,真皮毛囊减少,血管毛囊周围淋巴细胞浸润,部分淋巴细胞侵入毛囊内,部分细胞轻度异型。免疫组化示CD45RO、CD3、CD4和CD5阳性,CD20和CD30阴性。阿新蓝染色:毛囊内无粘蛋白沉积。皮损T细胞受体(TCR)重排阳性。结合临床、组织病理及免疫组化检查诊断为不伴粘蛋白沉积的嗜毛囊性蕈样肉芽肿。     

间质性蕈样肉芽肿一例

患者女,36岁,因躯干、四肢红斑、鳞屑伴瘙痒3年就诊。皮肤科检查：患者胸腹部、臀部可见边界不清的淡红斑,其上覆层状白色鳞屑。皮损组织病理学检查：真皮浅层单一核细胞灶状浸润,部分细胞有亲表皮性,亲表皮细胞有轻度异形,真皮中层胶原纤维束间可见单一核细胞呈间质性浸润和少许黏蛋白沉积。真皮浅中层散在浸润的单一核细胞均阳性表达T辅助细胞免疫表型。诊断：间质性蕈样肉芽肿。     

疱疹样天疱疮1例

患者男,25岁。头皮红斑脱屑1年伴躯干四肢红斑水疱、瘙痒1月。体检:头皮、躯干及四肢伸侧呈环形或半环形排列的紧张小水疱,尼氏征阴性。皮损组织病理示:表皮内水疱及嗜酸性海绵形成,无棘层松解细胞,真皮混合炎症细胞浸润。直接免疫荧光示C3棘细胞间沉积。诊断:疱疹样天疱疮。     

毛囊黏蛋白病二例并文献复习

报道2例毛囊黏蛋白病并进行文献复习.患者1,男,44岁,颈项部局限性浸润性红斑伴疼痛2个月,组织病理示:真皮毛囊水肿,毛囊内外淋巴组织细胞浸润,个别嗜酸粒细胞.阿新蓝染色:毛囊黏蛋白阳性.患者2,男,32岁,左面部浸润性暗红斑45天,组织病理示:表皮大致正常,毛囊上皮水肿,淡蓝色黏液样物质沉积,毛囊内及毛囊周围较多嗜酸...     

泛发性光泽苔藓并发毛发扁平苔藓

报告1例泛发性光泽苔藓并发毛发扁平苔藓。患儿女,4岁。头皮、颈项部、背部及四肢出现肤色丘疹4年余。皮肤科检查:头皮可见毛囊性针头大丘疹,毛发稀疏卷曲;颈项、背部及四肢可见针头大肤色坚实丘疹,有光泽。皮损组织病理检查:角化过度伴角化不全,基底细胞液化变性,真皮乳头浅层半球形浸润性细胞团块,团块两侧表皮突延长,呈典型的"抱球状"外观;同时可见毛囊上皮基底细胞液化变性,周围可见淋巴细胞为主的带状浸润。免疫组化显示浸润细胞以CD4~+细胞为主。诊断:泛发性光泽苔藓并发毛发扁平苔藓。治疗:给予他克莫司软膏,维A酸乳膏外用,转移因子口服液治疗,病情好转。     

肿胀性红斑狼疮误诊为过敏性皮炎

报告1例肿胀性红斑狼疮。患者女,24岁。面部反复出现红斑、斑块1年半,加重10 d。皮肤科检查:双侧面颊红斑、斑块,无鳞屑、瘢痕及萎缩。皮损组织病理检查:表皮角化过度,基底层及毛囊上皮细胞液化变性,血管及毛囊周围淋巴细胞及组织细胞浸润。阿辛蓝染色示真皮网状层黏蛋白沉积。根据其临床、皮损组织病理及免疫病理表现,诊断为肿胀性红斑狼疮。     

伴抗基底膜自身抗体的皮肤异色症样淀粉样变病1例

报道1例伴抗基底膜自身抗体阳性的皮肤异色症样淀粉样变病,并对相关文献进行复习.患者女,22岁.面颈部、躯干、四肢出现瘙痒性红斑、水疱及弥漫性网状色素沉着性斑片8年余.皮损组织病理示：表皮角化过度,基底细胞液化变性,真皮乳头均一化物质沉积,结晶紫染色阳性;直接免疫荧光（DIF）示：基底膜带及乳头IgG、IgM、IgA和C3沉积.     

首次命名淀粉样变型类天疱疮并附一例报道

本文首次提出淀粉样变型类天疱疮的概念，并报道一例。患者中年女性，躯干、四肢苔藓样皮疹伴瘙痒17年，无水疱。组织病理示：真皮乳头大量嗜酸性无定形物质沉积，结晶紫染色：阳性；DIF：真皮乳头及基底膜IgG、IgM颗粒状沉积，C3、IgA阴性；IIF：类天疱疮循环抗体IgG 1∶320阳性。人皮肤盐裂IIF：循环抗体IgG表皮侧阳性；抗BP180抗体0 U/mL，抗BP230抗体169.2 U/mL。     

Paraneoplastic pemphigus and Castleman's disease in the setting of herpes simplex virus infection

A 14-year-old girl presented with a 3-week history of mucosal erosions, injected conjunctiva, dehydration, and respiratory distress. She had been treated with intravenous acyclovir for herpes simplex infection with positive herpes simplex virus immunoglobulin M and immunoglobulin G. Physical examination and imaging revealed a large abdominal mass. Incisional biopsy was obtained, and pathology demonstrated angiofollicular hyperplasia with hyalinized germinal centers and Castleman's syndrome-like features. Based on the mucosal erosions, herpes simplex virus serology and positive herpes simplex virus-1 direct fluorescent antibody, Castleman's disease secondary to overwhelming herpes simplex virus infection was the initial impression. The poor response to antivirals and subsequent development of a bullous eruption on the hands resulted in dermatology consultation. Skin biopsy was obtained from a bullae and revealed suprabasilar acantholysis with necrosis as well as upper dermal, perivascular, and interface infiltrate of lymphocytes and eosinophils. No viropathic changes were present. Direct immunofluorescence was significant for immunoglobulin G deposition intercellularly and along the dermoepidermal junction and focal trace C3 deposition along the dermoepidermal junction consistent with paraneoplastic pemphigus, later confirmed by indirect immunofluorescence. We report this case of paraneoplastic pemphigus secondary to Castleman's syndrome confounded by herpes simplex virus-1 positive mucosal erosions.     

Unilateral colloid milium: a rare presentation

We report a case of a 31-year-old man with asymptomatic papules on one side of his face that had been present for 31 months. On physical examination, he had clusters of 1 to 4 mm skin colored translucent and dome-shaped, papules forming a plaque with a size of 4 x 3 cm on his left mandibular angle. Histopathology revealed atrophy of the epidermis with extensive deposition of pale eosinophilic homogenous deposits containing artifactual fissures that were PAS positive, diastase resistant, and Congo red negative. The final diagnosis was unilateral colloid milium.     

Paraneoplastic pemphigus associated with non-Hodgkin lymphoma

We report a case of a 55-year-old man who, after a 6-month history of enlargement of cervical lymph nodes, presented with multiple painful ulcerations of the oral mucosa and lips and multiple skin erosions on the trunk, back, extremities, and genitals. A lymph node biopsy was performed and revealed diffuse peripheral B-cell non-Hodgkin lymphoma. Skin biopsy revealed an acantholytic blister in the epidermis. Direct immunofluorescence showed IgG deposition in the intercellular spaces of the epidermis and linear C3 deposition in the basement-membrane zone. The indirect immunofluorescence test on rat urinary bladder epithelium was positive with a 1:320 titre. Paraneoplastic pemphigus was diagnosed based on these findings; treatment was started with cyclophosphamide, doxorubicin, vincristin and prednisone. The patient's response to treatment was poor and he developed several complications and died 2 months after diagnosis.     

Systemic sclerosis-like lesions during long-term penicillamine therapy for Wilson''s disease

Systemic sclerosis-like lesions developed in a 14-year-old boy with Wilson's disease who had been treated with D-penicillamine for 11 years. Clinical and laboratory manifestations included proximal scleroderma, pulmonary restrictive defects, positive antinuclear antibodies, and the deposition of C3 at the dermal-epidermal junction of the lesional skin. This is the first case reported in which long-term administration of penicillamine was followed by the development of systemic sclerosis-like lesions.     

黏液性汗管化生

报告1例黏液性汗管化生。患者男,52岁。左足跟内侧外伤后出现丘疹10年。皮肤科检查:左足跟内侧一直经约0.8 cm的丘疹,表面有糜烂和轻度渗液。皮损组织病理检查:表皮不规则增生,局部可见腺腔样分化,并可见杯状细胞。PAS及阿新蓝染色示杯状细胞染色阳性。免疫组化染色结果示腺腔及杯状细胞Cam5.2、癌胚抗原(CEA)及上皮膜抗原(EMA)阳性。诊断:黏液性汗管化生。     

Pemphigoid nodularis: a report of three cases and review of the literature

Many variants of bullous pemphigoid have been reported, some of which are clinically very atypical. One of the rarest variants is pemphigoid nodularis. We report three patients with this distinct entity. In all three cases patients initially presented with prurigo-like lesions and in one case nodular lesions preceded the onset of blistering by many months. Direct immunofluorescence was positive in all cases, with linear basement membrane zone deposition of IgG and C3. Circulating IgG antibasement membrane antibody was detected in two cases. and in these two patients on salt-split immunofluorescence the antibodies bound to the roof of the bullae.     

Reticular erythematous mucinosis syndrome: a case report

A 71-year-old man visited us with asymptomatic erythematous patches of two years' duration on the sternum and right costal arch areas. Histologically there was an appreciable amount of lymphocytic infiltration around the infundibulum of the hair follicle and the dermal capillaries, but the deposition of a colloidal iron positive material was observed around the lymphocytic infiltration and between the collagen bundles to only a mild degree. Reticular erythematous mucinosis (REM) syndrome is controversial; what is its nature as a mucinosis? This case seems to be the extreme of REM syndrome in which mucin deposition was minimal.     

Ichthyosiform mycosis fungoides with alopecia and atypical membranous nephropathy

We describe here a rare case of variant of mycosis fungoides (MF): ichthyosiform MF with alopecia and atypical membranous nephropathy. The diagnosis was made based on the following findings: generalized ichthyosis-like eruption, alopecia, enlarged superficial lymph nodes, proteinuria, and hematuria, the histological features of the skin biopsy from both ichthyotic and alopecic lesions with immunohistochemical staining, and the renal biopsy examination with immunofluorescence. The histological examination of ichthyotic and alopecic lesions displayed a predominant infiltration of atypical lymphocytes in the upper dermis with the characteristics of epidermotropism and folliculotropism. Immunohistochemical studies demonstrated that most infiltrated atypical lymphocytes were CD3, CD4, and CD45RO positive, whereas negative for CD5, CD7, CD20, CD30, and CD56. A renal biopsy examination revealed atypical membranous nephropathy with deposition of immunoglobulin G (IgG), IgM, IgA, C1q, and C3. In this case atypical membranous nephropathy was involved, which is very uncommon and has never been presented in the literature to date. Although ichthyosiform MF usually features a relatively favorable course, diffuse alopecia and the renal involvement in this case might indicate aggressive disease and poor prognosis.