麻风神经脓疡一例

患者,男,47岁。麻风确诊后未规则服药,神经炎症状突然加重,尺神经明显粗大（+++）,质硬,扪及杏仁大小皮下包块,触痛明显。     

误诊为银屑病的麻风一例

患者,男,25岁。全身暗红色斑片、斑块3个月,部分皮损上覆鳞屑,Auspitz征阳性。曾按“银屑病”治疗未好转。组织病理特征符合瘤型麻风,抗酸染色(+)。组织液涂片查抗酸杆菌:阳性。给予多菌型联合抗麻风药物,治疗1个月后皮损好转。     

Leprosy in a renal transplant recipient: a case report and literature review

Leprosy is rarely seen in organ transplant patients; only ten cases of leprosy in organ transplant recipients have been reported. We herein report a Taiwanese renal transplant recipient concomitantly infected with borderline lepromatous leprosy. A 68-year-old male received renal transplantation at Guilin, China, in 2000, and then received immunosuppressive therapy with prednisolone, tacrolimus, and mycophenolate. Three years after transplantation, multiple erythematous tender nodules and plaques over the face and lower limbs developed. Biopsies and histopathological examination confirmed the diagnosis of leprosy. We treated the patient with a multidrug regimen including dapsone, clofazimine, and rifampine since November of 2003 with a good response. Unfortunately, he suffered from a cluster of complications after an accidental fall, finally leading to septic shock and death five months later. In summary, we report a rare case of new-onset leprosy after renal transplantation in Taiwan and suggest leprosy should be listed in the differential diagnosis of unusual skin manifestations in organ transplant patients.     

Leprosy with pellagroid features (a case report)

A case of leprosy with pellagroid features is presented. Diagnosis of leprosy was confirmed by bacteriological and histopathological examination. A therapeutic trial with nicotinic acid did not result in any improvement. Occurrence of scaling in leprosy is discussed.     

麻风患者治愈后41年复发一例

患者,男,73岁。麻风治愈后41年,突发全身疼痛性红斑,躯干、四肢散在大小不一红色结节,伴全身乏力不适,面部浮肿等症状,无关节痛,无神经肿大疼痛。病理检查确诊麻风复发。立即上报麻风确诊病例,并申请联合化疗相关药物进行治疗,目前患者病情稳定,恢复良好,在麻风院村内继续观察治疗。     

以手足肿胀疼痛为首发症状的麻风一例

报告一例以手足肿胀疼痛为首发症状，皮损表现为四肢和躯干多处环状红斑的多菌型麻风。通过传统的方法和新兴的分子生物学方法对本例患者确定诊断，疗前检测氨苯砜综合征风险因子HLA-B*13:01阴性,给予MDT-MB治疗。     

Leprosy lesion on the prepuce of the male genitalia: a case report

A case of borderline leprosy in type I reaction with cutaneous lesions on the prepuce is reported. The need to examine the genitalia in all male leprosy patients is stressed.     

Leprosy acquired by inoculation from a knee injury

This case study reports on the development of clinical leprosy in a young Caucasian female from a non-endemic country who contracted the disease while living in a leprosy endemic country. In the presentation and discussion, some relevant factors will be reviewed and discussed that may play a role in the transmission, susceptibility and clinical development of the disease.     

获得性大疱性表皮松解症误诊一例并文献复习

报道一例反复误诊的获得性大疱表皮松解症并对相关文献进行复习。患者,女,25岁。皮疹泛发全身,主要表现为张力性水疱,疱壁紧张,尼氏征阴性,在外院误诊为天疱疮、线状IgA大疱病。组织病理检查示:表皮下水疱;盐裂IIF：IgG沉积在真皮侧; DIF:表皮基底膜IgG、C3、IgM、IgA带状沉积,ELISA：BP180,BP230均阴性,诊断为获得性大疱性表皮松解症,给予甲泼尼龙、氨苯砜、人免疫球蛋白、吗替麦考酚酯等治疗,病情好转。     

Lichen planus related to transforming growth factor beta inhibitor in a patient with metastatic chondrosarcoma: a case report

Transforming growth factor-beta1 (TGF-β1) is expressed in normal epidermis. TGF-β1 potently inhibits keratinocyte proliferation and immunomodulatory properties, mainly by suppressing immune responses to self-antigens. Lichen planus (LP) is a form of dermatitis caused by cell-mediated immune dysfunction, but the exact pathogenic pathways are unknown, which poses therapeutic challenges. We report on a 68-year-old man who developed multiple pruritic, discrete, and well-demarcated, flat-topped red-purple papules and macules on the back and upper arms following 4 cycles of treatment with TGF-β receptor I (TGFBR-I) inhibitor, ly3200882, for metastatic chondrosarcoma. The biopsy showed hyperkeratosis, wedge-shaped hypergranulosis, elongation of the rete ridges, and a dense band-like lymphohistiocytic infiltrate admixed with colloid bodies and pigment incontinence, consistent with LP. Temporal correlation suggested that the TGFBR-I inhibitor might be a trigger. Treatment with topical clobetasol and oral metronidazole led to partial resolution of the lesions with postinflammatory hyperpigmentation. We believe this is the first reported case of LP related to TGFBR-I inhibitor therapy. This report expands the list of cutaneous adverse events associated with this novel class of targeted therapy. More importantly, this report supports emerging evidence that failure of TGF-β1 activation/signal transduction is an important mechanism in the pathogenesis of LP and suggests the TGF-β1 pathway as a potential therapeutic target in this disease.     

Cyclosporine controls epidermolysis bullosa acquisita co-occurring with acquired factor VIII deficiency

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a rare antibody-mediated autoimmune blistering disease of adults. Likewise, acquired factor VIII deficiency is a rare antibody-mediated disease of adulthood. Both diseases can be exceedingly difficult to treat and refractory to immunomodulatory therapies. METHODS: Herein, we report a challenging case of EBA and acquired factor VIII deficiency in the same patient. RESULTS: Cyclosporine 4 mg/kg/day rapidly controlled both disease processes after the patient failed to respond to prednisone, colchicine, and pulse cyclophosphamide. The EBA relapsed when the cyclosporine was decreased to 2 mg/kg/day and the patient wore snuggly fitting new shoes, but it cleared quickly when the dose was increased. Cyclosporine has since been decreased gradually to 1.5 mg/kg/day without relapse of either condition or detectable side-effects. CONCLUSIONS: Cyclosporine should be considered when EBA and/or acquired factor VIII deficiency fail to respond to conservative therapy. Both conditions usually respond rapidly to cyclosporine 4-6 mg/kg/day.     

Adult autoimmune enteropathy presenting initially with acquired Acrodermatitis Enteropathica: a case report

Background

Acrodermatitis enteropathica (AE) is a rare dermatitis secondary to zinc deficiency most commonly seen as an inherited disease in infants. In the last decade, increased number of reports have been published on the acquired form that presents in adulthood. Unlike its inherited counterpart, acquired AE (AAE) is often secondary to underlying pathologic or iatrogenic etiologies that interfere with nutritional absorption, such as inflammatory bowel disease or alcoholism. Various gastrointestinal pathologies have been associated with AAE, but there is currently no report on its association with adult autoimmune enteropathy (AIE), a rare gastrointestinal disorder commonly seen in infants, with limited cases reported in adults. Here we present a case in which AAE was the initial clinical manifestation in an adult patient subsequently diagnosed with AIE.

Case presentation

A 41-year-old African American female presented to our emergency department at the Johns Hopkins Hospital with several months of progressively worsening dermatitis in the legs and acral regions, along with worsening symptoms of diarrhea, alopecia, poor oral intake, lethargy, hematochezia, peripheral edema, and weight loss. Our dermatology team was consulted given a presentation of exquisitely tender, erythematous, and diffusely desquamating skin lesions in the setting of two prior outside hospitalizations in the last 3 months with the same dermatitis that was refractory to topical and oral corticosteroids. Low serum zinc level and positive response to zinc supplementation confirmed the diagnosis of AAE. However, persistent hypovitaminosis and mineral deficiency despite aggressive nutritional supplementation prompted further investigation for an underlying malabsorption etiology. Jejunal biopsy and associated autoantibodies confirmed a diagnosis of adult AIE.

Conclusion

This case highlights the fact that adult AIE can present initially with clinical findings of AE. While proper zinc supplementation can resolve the latter, recognizing this association can trigger earlier diagnosis, minimize unnecessary tests, and establish earlier intervention to improve quality of life and prevent recurrence of AAE. The case also highlights the importance of collaboration between general and subspecialist physicians in identifying a primary etiology to a secondary clinical presentation. This report can be beneficial to general internists and emergency physicians, as much as it can be to dermatologists, rheumatologists, and gastroenterologists.

     

Leprosy treatment during pregnancy and breastfeeding: A case report and brief review of literature

Leprosy is a chronic disease which primarily affects the skin, mucous membranes and peripheral nerves due to Mycobacterium leprae. It is now infrequent in Europe and is rarely reported during pregnancy. Leprosy can be exacerbated during pregnancy, and without treatment it can permanently damage the skin, nerves, limbs and eyes. Therefore, it is important to treat leprosy during pregnancy. This article describes a patient with multibacillary lepromatous leprosy who was treated with multidrug therapy during pregnancy and breastfeeding. The patient delivered a healthy baby girl without perinatal complications, and the infant's growth and development were normal during the 1‐year follow‐up period. Multidrug therapy consisting of dapsone, rifampicine, and clofazimine is highly effective for people with leprosy and considered safe, both for the mother and the child. Antileprosy drugs are excreted into human milk but there is no report of adverse effects except for skin discoloration of the infant due to clofazimine. Multidrug therapy for leprosy patients should be continued unchanged during pregnancy and breastfeeding.     

获得性皮肤松弛症

报告1例获得性皮肤松弛症。患者女,17岁。面颈、双腋窝及股部皮肤松弛2年。皮肤科检查:面颈、双腋窝及股部皮肤明显松弛,皱纹密集,皮沟加深,无弹性。左颈部皮损组织病理示:表皮厚度大致正常,网篮状角化,真皮浅层血管周围少量淋巴细胞浸润。弹性纤维染色示:真皮浅层弹性纤维数量明显减少、形态异常。诊断:获得性皮肤松弛症。     

酪氨酸激酶抑制剂帕唑帕尼引起毛发变白一例

患者男,23岁,因头发胡须变白1个月就诊。患者自诉1个月前,部分胡须、头发根部同时变白,无自觉症状。追问病史,2010年6月,因右上颌窦间叶性软骨肉瘤。在全麻下行右上颌骨恶性肿瘤扩大切除 + 右上颌骨全切及生物膜植入术,术后予盐酸吉西他滨针及贝伐珠单抗注射液化疗。2个月前,发现右肩胛骨疼痛伴肿瘤骨转移,加用酪氨酸激酶抑制剂帕唑帕尼片800 mg/d口服。服药后1个月出现胡须、头发变白。否认早年白发家族史……     

Coexistence of pemphigus foliaceus and acquired hemophilia A: A case report

Pemphigus foliaceus (PF) is an autoimmune bullous dermatosis with anti‐desmoglein‐1 autoantibodies. Acquired hemophilia A (AHA) is a rare coagulation disorder with a high mortality rate, caused by anti‐factor VIII immunoglobulin G antibodies leading to spontaneous severe hemorrhages into skin, muscles or soft tissues. This coagulopathy may be associated with malignancies, drug reactions and autoimmune disorders including bullous dermatoses. Herein, we demonstrate a first report of AHA in the course of pemphigus foliaceus. A 55‐year‐old woman presenting with extensive, erosive, crusted, scaly skin lesions was diagnosed with PF based on histopathological and immunofluorescent examination, confirmed by the presence of anti‐desmoglein‐1 antibodies on enzyme‐linked immunoassay. She developed extensive internal hemorrhages and prolonged external bleeding after laparotomy. Based on coagulation tests, AHA was diagnosed. Simultaneous remission of pemphigus and coagulopathy occurred with immunosuppressants and recombinant activated factor VII.     

免疫检查点抑制剂帕博利珠单抗诱发银屑病一例并文献复习

报道1例57岁男性肺癌患者应用帕博利珠单抗后诱发银屑病并复习相关文献。临床表现为斑块型银屑病合并掌跖脓疱病和严重甲受累,既往无银屑病史或家族史。组织病理符合银屑病。经阿维A联合窄谱UVB治疗后病情控制。回顾文献,共有25例免疫检查点抑制剂诱发银屑病的报道。大多数患者为老年男性,以肺癌和黑素瘤为主,最常报道的诱发药物为纳武利尤单抗和帕博利珠单抗,从首次用药到出现皮损的中位时间为9周,临床表现以斑块型银屑病为主,组织病理特点与经典银屑病类似,所有患者经外用药、光疗和/或系统治疗后皮损改善,大部分患者无需停用免疫检查点抑制剂。