简介：AbstractIntroduction:Behçet disease is a chronic multisystem vasculitis disease, however generalized polymorphous cutaneous lesions are uncommon.Here, we reported a case of Behçet disease with multiple parts of the body and complex lesions, which may lead to misjudging in clinical diagnosis.Case report:A 69-year-old man presented with a seven-year history of recurrent generalized polymorphous mucocutaneous lesions (erythematous papules, nodules, ulcers, and necrosis) over his entire body, and the lesions had been painful for the past three years. Based on the past medical history, clinical presentation, histological examination excluded other diseases, and the 2014-amended International Criteria for Behget disease, the patient had a score of 6 points and was diagnosed as Behçet disease.Discussion:The common clinical feature in patients with Behçet syndrome is the presence of recurrent and usually painful mucocutaneous ulcers. Other clinical manifestations of this disorder are more variable among different patients. A diagnosis of generalized polymorphous cutaneous lesions should remain on the list of differential diagnosis of Behçet disease after excluding other diseases.Conclusion:The mucocutaneous lesions of Behçet disease are often preceded by other manifestations, and timely diagnosis may benefit early treatment and prognosis.

简介：AbstractAdult T-cell leukemia/lymphoma (ATLL) is an aggressive peripheral T-cell lymphoma caused by the human T lymphotropic virus type-1. The skin is affected in approximately half of ATLL patients, and skin lesions may be the first manifestation of the disease. The skin lesions of ATLL are polymorphous, and depend on the type of skin eruption, which makes it possible for doctors to predict the prognosis of the disease based on the characteristics of skin lesions. In this review article, we describe the clinical manifestations and histopathological patterns of skin lesions in ATLL, focus on its diagnostic and prognostic significance, and also summarize the advances in the treatment of ATLL.

简介：AbstractObjective:Skin diseases are common and striking features of patients with human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) and may vary considerably by ethnic and geographic regions and by the influence of highly active antiretroviral therapy (HAART). However, little information exists regarding the cutaneous manifestations of patients with HIV/AIDS in Bangladesh. This study was performed to elucidate the spectrum of cutaneous disorders in patients with HIV/AIDS in the era of HAART.Materials:This descriptive cross-sectional study was carried out in Chittagong Medical College Hospital, Bangladesh from January 2017 and December 2020. Diagnosed case of HIV/AIDS for HAART therapy and all cases of HIV/AIDS who are already on HAART therapy were included in this study. Descriptive statistical analysis was carried out by using frequencies and percentages.Results:Of 40 patients with HIV/AIDS, 22 (55.0%) were male and 18 (45.0%) were female. The patients ranged in age from 8 to 60 years, with a mean age of 38 ± 0.966 years. Among all age groups, the highest 19 (47.5%) patients were in the 31- to 40-year age group. Most of the patients were migrant workers [22/40 (55.0%)] with low socioeconomic status [32/40 (80.0%)], and the most common transmission mode was heterosexual activity [36/40 (90.0%)]. Most of the patients [32/40 (80.0%)] had mucocutaneous disorders, 30/40 (75.0%) had infective dermatoses, and 21/40 (52.5%) had non-infective inflammatory dermatoses. Eight of forty (20.0%) patients presented with three or more skin disorders. The most common infective dermatoses were fungal infections [15/40 (37.5%)], followed by viral infections [8/40 (20.0%)], bacterial infections [4/40 (10.0%)], and scabies [3/40 (7.5%)]. The most common non-infective dermatosis was generalized pruritus [6/40 (15.0%)], followed by prurigo simplex [4/40 (10.0%)], psoriasis [4/40 (10.0%)], eczema [3/40 (7.5%)], pruritic papular eruption [1/40 (2.5%)], seborrheic dermatitis [1/40 (2.5%)], urticaria [1/40 (2.5%)], and xerosis [1/40 (2.5%)]. Patients treated with HAART had decreased rates of oral candidiasis and herpes simplex but increased rates of drug reactions [19/40 (47.5%)]. The most common drug eruption following HAART was a morbilliform rash [11/40 (27.5%)], and the most common offending agent was nevirapine. The prevalence of mucocutaneous disorders was higher in patients with a CD4 cell count of <200 cells/mm3.Conclusions:A wide range of mucocutaneous disorders is observed in Bangladeshi patients with HIV/AIDS, and HAART has an impact on the spectrum of HIV/AIDS-associated mucocutaneous disorders. Skin and mucocutaneous disorders are seen at every stage of HIV/AIDS and are the initial presentation in most patients in Bangladesh. There is a need for increased attention to the diagnosis and treatment of skin diseases affecting the quality of life of patients with HIV/AIDS.

简介：摘要本文报道1例发生于女性儿童的laryngo-onycho-cutaneous综合征（LOCS）。患儿女，4岁，因“出生后反复声音嘶哑、喉鸣音4年余，加重2个月”于2020年10月27日在上海交通大学医学院附属上海儿童医学中心耳鼻咽喉科就诊。体格检查示全身散在皮疹及溃疡样皮肤缺损，双侧脚趾甲及双手指甲部分缺如，牙釉质发育不良及牙齿异常等表现。电子喉镜检查发现声门上区肉芽组织增生明显，声门下狭窄，双侧声带活动受限。基因检测结果提示患儿LAMA3基因存在复合杂合变异c.151dupG（p.Val51Glyfs*4）和c.3216G>A（p.Ser1072Ser），其父母分别各携带LAMA3基因的一处杂合变异。结合患儿的临床表型、电子喉镜表现以及全外显子组检测结果，该患儿被诊断为LOCS。因父母拒绝进一步治疗，未给予干预措施，密切随访中。

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