皮肤痘疮样斑状萎缩一家系

皮肤痘疮样斑状萎缩(A trophiam aculosa varioliform is cutis,AM V C)是一种少见的遗传性皮肤病(OM IM601341)。本病病因和发病机制至今不明,文献中大部分为散发病例。最近我院诊断一例皮肤痘疮样斑状萎缩,其家族成员中有类似疾病的患者,我们进行了家系临床资料的采集,现报道     

皮肤痘疮样斑状萎缩一例

患儿女,4个月,以躯干、四肢凹点状皮肤萎缩3个月就诊。患儿3个月前无明显诱因躯干、四肢起较多绿豆至豌豆大淡灰褐色斑疹。皮损发生前无红斑,基底无浸润及潮红。皮损在短期内即发生凹点状萎缩,颜色转为苍白。     

急性痘疮样苔藓样糠疹1例

1临床资料 患者男,32岁,躯干、四肢皮疹半年来诊.患者半年前无明显诱因躯干、四肢起红斑、小丘疹,上覆细薄鳞屑,部分丘疹顶端水疱、破溃,形成溃疡、结痂,痂皮脱落后遗留瘢痕,皮疹成批发生.曾在外院诊断为湿疹,用中药治疗(具体不详)效果不佳.家族中无类似病史.体格检查:体温36.4℃,脉搏73次/min,呼吸20次/min,血压15.6/10 kPa.一般情况可,系统检查无异常.皮肤科检查:躯干、四肢散在分布的鳞屑性暗红色、紫红色斑丘疹、丘疹、丘疱疹,上覆鳞屑不易剥离,剥离后无薄膜现象及点状出血;红斑直径约1～4mm,丘疹、丘疱疹约粟粒至绿豆大小,部分皮疹中央有坏死或溃疡,个别皮疹愈后有色素沉着及痘疮样凹陷,见图1.     

急性痘疮样苔藓样糠疹1例

1　病例　患者男 ,32岁 ,干部。因躯干、四肢分批出现坏死性丘疹 2月就诊。 2月前患者在无明显诱因的情况下双上臂、前胸及后背出现散在淡红丘疹 ,无明显不适感。以后皮损渐多 ,皮损累及腹部及双大腿 ,同时部分丘疹发生水疱出血 ,坏死及结痂。曾自用皮炎平 ,皮康王等。病情未见好转。故来本科就诊。体检 :一般情况良好 ,各系统检查未见异常。皮肤科情况 :躯干、四肢可见散在分布的淡红、红褐。红黑色豌豆大的圆形丘疹 ,有出血、坏死及结痂。表面附以细屑、皮损以双上臂及胸背部多见。口腔粘膜及外生殖器未见类似皮损。实验室检查 :血尿常规…     

原发性斑状萎缩1例

患者女,52岁。躯干、上肢出现丘疹、萎缩性斑片,逐渐增大、增多16年。皮损组织病理示:表皮萎缩变薄,真皮浅层血管扩张,血管周围散在以淋巴细胞为主的炎性细胞浸润,真皮胶原纤维排列紊乱,部分断裂,胶原间隙增宽;弹性纤维染色可见皮损处弹性纤维明显减少和断裂。诊断:原发性斑状萎缩。     

Schweninger-Buzzi型原发性皮肤松弛症1例

皮肤松弛症又称斑状萎缩,是以真皮正常弹性纤维明显减少或丧失、在皮肤发生局限性圆形或椭圆形萎缩松弛的疝样斑为特征,临床少见,分为原发和继发性2类.本文报道1例Schweninger-Buzzi型皮肤松弛症.     

原发性斑状萎缩1例

斑状萎缩是一种以真皮弹性纤维减少导致表皮松弛,并呈囊状的少见皮肤病,可以继发于狼疮和抗磷脂抗体综合征的自身免疫性疾病。无明显病因者称为原发性斑状萎缩。本文报告1例。     

进行性特发性皮肤萎缩1例

进行性特发性皮肤萎缩又称Ｐａｓｉｎｉ Ｐｉｅｒｉｎｉ特发性皮肤萎缩 ,是一色素性皮肤萎缩性疾病 ,现将所见 1例报告如下。临床资料　患者 ,女 ,31岁。因左前臂、左小腿棕红斑半年 ,于 2 0 0 2年 1月 17日来我院就诊。半年前患者左前臂、左小腿无明显诱因出现数块棕红斑 ,不痛不痒 ,未做任何治疗 ,皮损不断蔓延、扩大 ,故来我院就诊。家族中无类似患者体检　一般情况好 ,系统检查未见异常。皮肤科情况 :左前臂屈侧、左小腿内侧散在数块棕红色萎缩性圆形、卵圆形或不规则形斑 ,直径约 2～ 10ｃｍ大小 ,界欠清 ,表面光滑 ,皮下血管隐约可见 …     

痘疮样水疱病1例

痘疮样水疱病是一种自幼发病，以水疱为主的特发性光敏性皮肤病，愈后遗留疤痕。本病临床较为少见，现报道1例如下。 患者女性，14岁，云南临沧人。暴露部位皮肤反复发生红斑、水疱、痒痛9年余。3岁时，因曝晒太阳致面部皮肤发生红斑水肿，伴瘙痒刺痛。随后，红斑上出现粉红色丘疹，继之在丘疹顶端发生水疱、血疱，血疱破后出血结痂。皮损于秋冬季好转，春夏季加重，自觉瘙痒疼痛。几年来反复发作，逐步发展到颈部、手背及足背，但无明显全身症……     

短期应用糖皮质激素致重症皮肤萎缩纹1例

1　临床资料　患者男 ,12岁。以下肢多发性条状暗红色斑 3个月就诊。半年前四肢出现丘疹、红斑、脱屑 ,伴瘙痒 ,在当地诊所诊断为银屑病 ,给予中成药消银片 2片、扑尔敏 4mg、VitC 0 .1g、强的松 5mg ,均 3次 /d口服 ,共用 10天 ;外用皮炎平霜 2次 /d ,共用 3支。 15天后皮损消     

A Case of Atrophia Maculosa Varioliformis Cutis

Atrophia maculosa varioliformis cutis (AMVC) is a type of idiopathic noninflammatory macular atrophy that occurs in young individuals. It is clinically characterized by shallow, sharply demaracated depressions of various shapes. Considering that atrophia maculosa varioliformis cutis can be mistaken as scarring and artifact dermatitis, it is important for physicians to distinguish this condition and to diagnose it correctly.     

Familial Atrophia Maculosa Varioliformis Cutis: An Ultrastructural Study

Atrophia maculosa varioliformis cutis is a rare and distinctive form of idiopathic facial macular noninflammatory atrophy that may rarely be observed in members of the same family. We describe two brothers, ages 14 and 16 years, with spontaneously appearing, asymptomatic, varioliform and linear atrophic lesions. Their past medical history was positive for varicella occurring in childhood without residual facial scarring. Routine laboratory investigations and screening for circulating autoantibodies were negative. Both patients were concordant for HLA A2 and DQ4.1. Routine and ultrastructural histologic examination of a punch biopsy specimen showed the presence of scarce, small, fragmented elastic fibers and compact collagen bundles associated with hypertrophic fibroblasts in the dermis. Our patients remained clinically stable, untreated, over a 2-year follow-up period. No long-term follow-up data have previously been reported.     

Familial atrophia maculosa varioliformis cutis: case report and pedigree analysis

Atrophia maculosa varioliformis cutis (AVMC) was first described in 1918, as a rarely reported form of idiopathic macular atrophy on the cheeks. Nineteen patients have been reported in the past 86 years. Recently we diagnosed a 25-year-old woman as AMVC and investigated her family history. We collected the clinical data of the pedigree and presumed that AVMV is in a autosomal dominant inheritance.     

Monozygotic twins with atrophia maculosa varioliformis cutis

Atrophia maculosa varioliformis cutis (AMVC) is a sporadic or inherited childhood disorder, signified by the occurrence of pitted scars, usually over the face. We report two cases of AMVC occurring in monozygotic twins.     

Solitary Milialike Idiopathic Calcinosis Cutis: A Case Unassociated with Down Syndrome

We report a unique case of solitary milialike idiopathic calcinosis cutis (MICC) in a healthy Korean woman, which is not associated with Down syndrome. This case of MICC would be a form of idiopathic calcinosis cutis, which can be solitary or multiple, sporadic or associated with Down syndrome.     

A Case of Congenital Leukemia Cutis

Congenital leukemia is a rare condition diagnosed at birth to 6 weeks of life with an estimated incidence of 4.7 per million live births. In a review, about 25~30% of cases were well documented as leukemia cutis. We report a case of a 3-month-old infant presented with asymptomatic multiple erythematous to bluish patches and nodules, which had developed about two months ago. Biopsy of the cutaneous lesions revealed immature cells which are overall monotonous with large kidney shaped nucleus, prominent nucleoli and moderate cytoplasm. These atypical cells stained positive for leukocyte common antigen, lysozyme and CD68. With this result, a congenital leukemia cutis was diagnosed. Six months later, she underwent cord blood stem cell transplantation. Seven months after the cord blood stem cell transplantation, the bone marrow examination revealed a normal result. Leukemia can be presented in the skin, the eruption may be nonspecific and it may precede systemic involvement. The recognition is important because early diagnosis can lead to appropriate treatment, followed by better prognosis.     

A Case of Congenital Leukemia Cutis

Congenital leukemia is a rare disease that develops from birth to 6 weeks of life. Leukemia cutis involves cutaneous infiltration by leukemic cells and is an unusual manifestation of leukemia, and has been documented in 25~30% of patients with congenital leukemia. The authors report a case of congenital leukemia cutis. A newborn male presented with widespread firm dusky red papules and nodules on almost his entire body surface. Skin biopsy specimens confirmed the presence of leukemic infiltrations, and bone marrow cytology was consistent with acute myeloid leukemia of the FAB M5 type.     

臀部疣状皮肤结核误诊1例

患儿女,10岁,右臀部环状斑块8余年,曾多次误诊为股癣。皮损组织病理示:表皮角化不全,毛囊角栓,表皮假上皮瘤样增生;真皮浅中层弥漫炎细胞浸润,以淋巴细胞为主;真皮内可见上皮样细胞结节,浸润的细胞有淋巴细胞、多核巨细胞及组织细胞等。抗酸染色(-),PPD试验(3+)。诊断:疣状皮肤结核。     

急性痘疮样苔藓样糠疹1例及文献复习

患者男,47岁,躯干、四肢皮肤见泛发粟粒至黄豆大红色斑丘疹20天,丘疹上覆少许稀薄鳞屑,Auspit征(-),部分皮损中央破溃,坏死、结痂。患者无发热、淋巴结及肝脾肿大。组织病理示:表皮呈灶性变性坏死,区域性淋巴浸润。诊断:急性痘疮样苔藓样糠疹。本文并就其病因、临床表现、病理进展和治疗进行了文献复习。     

A Case of Tuberculosis Cutis Orificialis with Perianal Involvement

Tuberculosis cutis orificialis (TCO) is a rare manifestation of cutaneous tuberculosis that is caused by auto-inoculation of mycobacteria in patients with advanced internal tuberculosis. TCO occurs in oral, perianal, or genital mucosa and adjacent skin. The tongue is the most frequently affected site, but the perianal area can also be affected. A 39-year-old male presented with a 4-month history of painful ulcers on the perianal area. The histopathologic findings revealed granulomatous infiltrates composed of epithelioid cells and Langhans-type giant cells in the dermis, and a few acid-fast bacilli noted on Ziehl-Neelsen staining. The polymerase chain reaction (PCR) was positive for Mycobacterium tuberculosis and the chest X-ray showed findings consistent with active pulmonary tuberculosis in both upper lung zones. The skin lesion showed complete resolution 2 months after the start of treatment with antituberculosis agents. We report a case of TCO with perianal involvement in a patient with underlying active pulmonary tuberculosis.