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Erlotinib-induced Perioral Lesions Resembling Scleroderma. 厄洛替尼诱发的类似硬皮病的口周病变
Constantin A Dasanu, Juliana Alvarez-Argote, Rossel G Dasanu, Abram Soliman, Ion Codreanu
<p><p>Erlotinib, an epidermal growth factor receptor tyrosine kinase inhibitor (EGFR-TKI), is currently used in the therapy of several solid malignancies. This agent has been associated with several dermatological side-effects, the most common being papulo-pustular acneiform rash. Herein we describe a unique skin effect in a patient treated with erlotinib for non-small cell lung cancer. A 68-year-old Caucasian woman presented with complaints of an unusual perioral rash associated with redness and pain. A "feeling of tension" that interfered with chewing was described by the patient. She denied myalgias, arthralgias, shortness of breath, or other skin lesions. The patient also denied self-infliction of wounds, physical abuse, or trauma. Three months before this presentation, she was diagnosed with metastatic lung adenocarcinoma in the liver and adrenal glands, which carried an exon 21 L858R EGFR-TK mutation. A month after the diagnosis, the patient was started on erlotinib 150 mg PO daily, which she had continued up to the time the perioral skin lesions commenced. Besides erlotinib, the patient denied any other new medications. At the start of perioral skin lesions, a restaging computed tomography (CT) scan showed a decrease in size of all metastatic site lesions, consistent with a partial response to erlotinib. Other comorbidities included peptic ulcer disease and hypertension. The patient had a 5-pack year history of tobacco smoking in her early 20s. Skin examination showed convergent erythematous perioral lesions with a cut-like appearance, some having healed with crusts and others with hyperkeratotic scars (Figure 1, A, B). Physical examination was further remarkable for a grade 1 papulo-pustular acneiform rash involving the face (Figure 1, B) and grade 1 xerosis of the face (Figure 1, A, B) and both hands. Complete blood count, comprehensive metabolic panel, C3 and C4 complement fractions, C-reactive protein, as well as antinuclear, anti-DNA, anti-protein-A and -B, and anti-SCL-70 antibodies were within normal range. The patient was advised to apply colloidal oatmeal lotion to the lesions three times per day. She reported a moderate improvement in skin lesions and decreased anxiety about their appearance. Erlotinib was continued at the same dose. The most recent re-evaluation CT scan showed a continued clinical response of lung cancer to erlotinib. Causality between EGFR-TKIs and various skin lesions is well-documented (1-3). The most commonly seen manifestation in this context is papulo-pustular, acneiform rash involving the face, neck, and torso, with an incidence of 70-80% (1). The dermatologic toxicity of erlotinib to the fingernails and distal phalanges includes xerosis, paronychia, and finger fissures (4). The skin toxicity of erlotinib is thought to be due its complex effects on keratinocyte growth and differentiation (5,6). The present patient's perioral deep, cut-like lesions penetrated into the deep dermis (Figure 1, A, B). Xerosis
厄洛替尼是一种表皮生长因子受体酪氨酸激酶抑制剂(EGFR-TKI),目前用于治疗几种实体恶性肿瘤。该药物与几种皮肤病副作用有关,最常见的是丘疹-脓疱性痤疮皮疹。在这里,我们描述了一个独特的皮肤效果,在病人接受厄洛替尼治疗非小细胞肺癌。一名68岁的白人妇女提出了一个不寻常的口周皮疹与红色和疼痛相关的投诉。病人描述了一种干扰咀嚼的“紧张感”。她否认有肌痛、关节痛、呼吸短促或其他皮肤病变。病人还否认有自我伤害、身体虐待或创伤。在这次报告前三个月,她被诊断为肝脏和肾上腺转移性肺腺癌,携带外显子21 L858R EGFR-TK突变。诊断一个月后,患者开始服用厄洛替尼150mg PO每日,她一直持续到口腔周围皮肤病变开始。除了厄洛替尼,病人拒绝使用任何其他新药。在口腔周围皮肤病变开始时,重新扫描计算机断层扫描(CT)显示所有转移部位病变的大小减小,与厄洛替尼的部分反应一致。其他合并症包括消化性溃疡和高血压。患者20岁出头有5年的吸烟史。皮肤检查显示收敛红斑的口周的如一把外观损伤,一些与角化过的疤痕愈合与外壳等(图1中,a、B),体检进一步显著年级papulo-pustular acneiform皮疹涉及(图1,B)和1级干燥病的脸(图1中,a, B)和两只手。全血计数、综合代谢组、C3和C4补体分数、c反应蛋白、抗核、抗dna、抗蛋白a和-B、抗scl -70抗体均在正常范围内。建议患者每日三次在患处涂抹胶体燕麦乳液。她报告说,皮肤损伤有了适度的改善,对皮肤外观的焦虑也减少了。厄洛替尼继续以相同剂量使用。最近的重新评估CT扫描显示肺癌对厄洛替尼有持续的临床反应。EGFR-TKIs与各种皮肤病变之间的因果关系已得到充分证明(1-3)。在这种情况下,最常见的表现是丘疹、脓疱、痤疮样皮疹,涉及面部、颈部和躯干,发生率为70-80%(1)。厄洛替尼对指甲和远端指骨的皮肤毒性包括干燥、甲沟炎和指缝(4)。厄洛替尼的皮肤毒性被认为是由于其对角化细胞生长和分化的复杂影响(5,6)。本例患者的口周深部切口样病变渗透到真皮深部(图1,A, B)。在这些病变发生之前可能已经发生了干燥,因为胶体燕麦洗剂改善了这些病变。然而,本例患者的一个独特特征是口腔周围的定位类似硬皮病(图1,a, B)。本例患者的病变提出了一个临床问题:她是在自残,还是她的伤口代表了厄洛替尼的副作用?虽然这些损伤有割伤的外观,但它们不符合非自杀性自伤(NSSI)(7)。患者否认有任何精神疾病史或自伤病史。此外,与自伤患者所见的损伤不同,伤口是双侧对称的。患者配合局部治疗,病灶也相应改善。我们假设厄洛替尼是导致所描述的深度,切口样病变的病因,因为它是当时患者服用的唯一新药物。她在服用厄洛替尼两个月后经历了这种效果。此外,患者还经历了该药的其他副作用,包括丘疹-脓疱性痤疮皮疹和干燥。此外,医学文献支持使用胶体燕麦乳液缓解EGFR TKIs引起的皮肤表现(8),这与本病例的积极反应一致。根据Naranjo药物不良反应概率量表,厄洛替尼与患者的硬皮病样病变之间的因果关系被评为可能的,得分为8分。厄洛替尼的这种皮肤毒性作用以前在医学文献中没有报道过。虽然一些作者表明,由于这类药物,其他皮肤效应的改善是剂量依赖性的(1),但在我们的病例中,不需要中断或减少厄洛替尼的剂量。 对厄洛替尼引起的硬皮病样病变的认识对于向患者提供保证,继续抗癌治疗,避免不必要的昂贵检查或转介给皮肤科或精神病学专家是很重要的。
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引用次数: 0
Ultra-high-frequency Ultrasound in the Objective Assessment of Chlormethine Gel Efficacy: A Case Report. 超高频超声在氯甲基凝胶疗效客观评价中的应用1例。
Agata Janowska, Cristian Fidanzi, Marco Romanelli, Alessandra Michelucci, Matteo Bevilacqua, Valentina Dini

Mycosis fungoides (MF) represent the most frequent form of cutaneous T-cell lymphoma (CTCL). Chlormethine gel has been approved as first-line therapy in MF. The classification of early forms of MF is clinically and histologically complex even for experienced clinicians. Ultra-high-frequency ultrasound (UHFUS) is a new non-invasive method that is capable of supporting clinical evaluation, thus providing both a diagnostic guide for distinguishing different stages of the disease and a therapeutic method in terms of monitoring the treatment. In our case report, we clinically and ultrasonographically evaluated the efficacy of chlormethine gel treatment on patches resistant to previous local treatments. A 63-year-old patient was treated for MF stage 1B with cycles of topical clobetasol cream and PUVA and UVB narrow-band phototherapy for 2 years, with a partial therapeutic response. Patches remained at the level of the left and right hips and lower legs. The patient began treatment with chlormethine gel 3 times a week for 2 months. We performed clinical and ultrasound evaluations on the left hip at baseline and after 2 months. The ultrasound examination was performed using (VEVO MD, VisualSonics), both in B-mode and C-mode, with a 70 MHz frequency probe. At baseline, we found a hypoechoic band of 0.94 mm named the Subepidermal Low Echogenity Band (SLEB), localized under the dermo-epidermal junction. Multiple dilated hair follicles with vascular invasion, disrupting the dermo-epidermal junction, and numerous vascular lacunae at the dermal level were found using C-mode. After 2 months of treatment, the lesions had clinically healed with the persistence of a pigmentary outcome. UHFUS identified minimal thickness of the SLEB and minimal vascularization. Chlormethine gel was an effective and well-tolerated treatment in patches unresponsive to other topical treatments. The use of a non-invasive diagnostic technique can allow objective assessment of lesions with a better therapeutic approach.

真菌病(MF)是最常见的皮肤T细胞淋巴瘤(CTCL)。氯甲胺凝胶已被批准作为 MF 的一线疗法。即使是经验丰富的临床医生,早期 MF 的临床和组织学分类也十分复杂。超高频超声(UHFUS)是一种新的无创方法,能够支持临床评估,从而为区分疾病的不同阶段提供诊断指导,并为监测治疗提供治疗方法。在我们的病例报告中,我们通过临床和超声波检查评估了氯甲胺凝胶治疗对既往局部治疗耐药斑块的疗效。一位 63 岁的患者因中耳炎 1B 期而接受了长达 2 年的局部氯倍他索乳膏和 PUVA 及 UVB 窄波段光疗,并取得了部分治疗效果。左右臀部和小腿处仍有斑块。患者开始使用氯甲胺凝胶治疗,每周 3 次,持续 2 个月。我们在基线和两个月后对左髋部进行了临床和超声波评估。超声检查使用的是 70 兆赫频率探头(VEVO MD,VisualSonics),包括 B 型和 C 型。基线时,我们在真皮-表皮交界处发现了一条 0.94 毫米的低回声带,名为表皮下低回声带(SLEB)。使用 C 型超声波检查发现,多处扩张的毛囊伴有血管侵犯,破坏了真皮-表皮交界处,真皮层出现大量血管裂隙。治疗 2 个月后,皮损临床痊愈,但仍有色素沉着。超高频超声波检查发现SLEB的厚度极小,血管也极少。对于其他局部治疗无效的斑块,氯甲胺凝胶是一种有效且耐受性良好的治疗方法。使用非侵入性诊断技术可以对病变进行客观评估,从而采取更好的治疗方法。
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引用次数: 0
Diagnosis, Investigation and Management of Non-immediate (Type IV) Cutaneous Adverse Drug Reactions. 非立即(IV型)皮肤药物不良反应的诊断、调查和处理。
Vesna Vukičević Lazarević

Pathophysiologically, drug hypersensitive reactions (DHRs) are classified into four types: type I, immediate reactions, and types II, III, and IV, non-immediate reactions. They are further categorized as severe or non-severe based on clinical severity. Genetic predisposition and viral reactivation are cofactors of severe DHR type IV. Rapid identification, cessation, and future avoidance of offending and cross-reactive drugs are the cornerstone of treatment. The diagnosis is established based on the history, physical examination, and subsequent in vivo, in vitro, and drug provocation testing, which are selected based on the severity of DHR. In non-severe DHRs, desensitization to the offending drug is possible.

在病理生理学上,药物超敏反应(DHRs)分为四种类型:I型,即立即反应,以及II、III和IV型,即非立即反应。根据临床严重程度进一步分为严重或非严重。遗传易感性和病毒再激活是严重DHR IV型的辅助因素。快速识别、停用和未来避免使用不良和交叉反应性药物是治疗的基石。诊断依据病史、体格检查以及随后的体内、体外和药物激发试验,根据DHR的严重程度进行选择。在非严重的dhr中,可能对致病药物脱敏。
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引用次数: 0
Blastic Plasmacytoid Dendritic Cell Neoplasm, a Very Rare Hematological Malignancy With Initial Cutaneous Involvement: A Case Report. 一种非常罕见的最初皮肤受累的血液恶性肿瘤--疱性浆细胞样树突状细胞肿瘤:病例报告。
Jelena Lalošević Mišović, Jovan Lalošević, Mirjana Gajić-Veljić, Tatjana Terzić, Miloš Nikolić

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare and aggressive hematologic malignancy, arising from plasmacytoid dendritic cells (pDCs). BPDCN frequently has, at least initially, exclusively cutaneous presentation. We present a 45-year-old woman with a 3-month history of rapidly evolving violaceous patches, infiltrated plaques, and bruise-like tumefactions, disseminated on her face and upper trunk. Histopathology of a lesion, along with Flow cytometry of peripheral blood and cerebrospinal fluid, confirmed the diagnosis of BPDCN. The patient received a hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone) regimen and six triple intrathecal chemotherapies. Unfortunately, the patient contracted COVID-19 and died of severe respiratory complications, despite all the available treatment modalities. Our patient presented with typical clinicopathological manifestations of the disease, misdiagnosed for 3 months. The case additionally reflects difficulties in patient management during the COVID-19 pandemic. Dermatologists should be aware of this rare disease, since the early diagnosis and treatment with new emerging drugs may lead to a better prognosis.

母浆细胞样树突状细胞肿瘤(BPDCN)是一种由浆细胞样树突状细胞(pDCs)引起的非常罕见的侵袭性血液恶性肿瘤。至少在最初,BPDCN通常只表现为皮肤。我们报告一名45岁的女性,3个月的病史,快速发展的紫色斑块、浸润性斑块和瘀伤样肿物,分布在她的面部和上躯干。病变的组织病理学,以及外周血和脑脊液的流式细胞术证实了BPDCN的诊断。患者接受了超cvad(环磷酰胺、长春新碱、阿霉素、地塞米松)方案和6次三联鞘内化疗。不幸的是,尽管采用了所有可用的治疗方式,患者还是感染了COVID-19,并死于严重的呼吸道并发症。我们的病人有典型的临床病理表现,误诊3个月。该病例还反映了COVID-19大流行期间患者管理的困难。皮肤科医生应该注意这种罕见的疾病,因为早期诊断和使用新兴药物治疗可能会导致更好的预后。
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引用次数: 0
Cystic Basal Cell Carcinoma with a Giant Vulvar Cyst. 伴有巨大外阴囊肿的囊性基底细胞癌。
Takayuki Suyama, Megumi Yokoyama, Jun Matsushima, Kazumoto Katagiri
<p><p>Cystic basal cell carcinoma (BCC) is a rare subtype of BCC (1). Histologically, it is usually characterized by multiple small cysts without a clinical cystic appearance (2). Herein, we report an unusual case of cystic BCC with a large vulvar cyst. A 90-year-old Japanese woman visited our hospital with a pedunculated subcutaneous nodule on her right labia majora that had persisted for 10 years and had grown rapidly in the past 4 years. The initial examination revealed a cystic tumor (size 90×70×60 mm) (Figure 1, A). Magnetic resonance imaging revealed a cystic mass surrounded by a focally thickened wall on the vulva (Figure 1, B), with T2 high and T1 low intensities in the center. Therefore, an epidermoid cyst or other type of cystic tumor was considered for diagnosis. The tumor was successfully excised with the overlying epidermis (Figure 1, C), the skin defect was primarily closed, and the deformity was corrected. During sample processing for pathological evaluation, brown serous fluid with no keratin leaked from the cyst, leading to cyst shrinkage (Figure 1, D). Histopathological evaluation revealed a thickened cyst wall and basaloid cells with peripheral palisading cell arrangements and slight atypia. Squamous epithelium with a granular layer and keratinization were absent (Figure 2, A), while mucin deposition was apparent in the tumor nests (Figure 2, B). Moreover, the walls of the cyst showed partial thinning (Figure 2, C). On immunohistochemistry, the tumor nests were negative for epithelial membrane antigen (EMA) (Figure 2, D), carcinoembryonic antigen, and gross cystic disease fluid protein 15. The mucin of the stroma was positive for Alcian blue stain (Figure 2, E). Thus, the tumor was diagnosed as a cystic BCC. No evidence of recurrence has been observed as of 20 months after surgery. BCCs that form single cysts, especially those completely composed of BCC cells or those that develop from epidermoid cysts, are very rare; however, in a few cases, the cyst walls comprised squamous cells with keratohyalin granules, and BCC cells were present in some parts of the tumor (3,4). In these cases, the tumor sizes were <50 mm (3,4). Our patient had a cystic BCC with a single cyst that contained serous fluid without keratin, and the cyst wall was completely composed of BCC cells. The tumor cells were negative for EMA (Figure 2, D). Whether the BCC in the present case originated from the overlying epidermis or from epidermoid cysts was unclear. Based on the complete lack of keratin and squamous epithelium in the wall of the cyst, the epidermoid cyst origin was less likely, despite a possible link with the overlying epidermis. However, no continuation between the cyst wall and the overlying epithelium was observed, which may reflect the large size of the tumor. Nevertheless, one section of the BCC was beneath the cyst (Figure 2, F), with a possible connection between the wall of the cyst and the overlying epidermis. Alternatively, the BCC may n
囊性基底细胞癌(BCC)是一种罕见的基底细胞癌亚型(1)。组织学上,它通常以多个小囊肿为特征,没有临床囊性表现(2)。在此,我们报告一例罕见的囊性基底细胞癌合并外阴大囊肿。一位90岁的日本妇女因右大阴唇有带蒂皮下结节就诊,该结节持续10年,近4年来生长迅速。初步检查显示囊性肿瘤(大小90×70×60 mm)(图1,a)。磁共振成像显示外阴处囊性肿块被局灶性增厚壁包围(图1,B),中心呈T2高、T1低强度。因此,可考虑诊断为表皮样囊肿或其他类型的囊性肿瘤。成功切除肿瘤及其覆盖的表皮(图1,C),皮肤缺损基本闭合,畸形得到纠正。在进行病理检查的样品处理过程中,囊肿渗出褐色浆液,不含角蛋白,导致囊肿缩小(图1,D)。组织病理学检查显示囊肿壁增厚,基底样细胞呈外周栅栏状排列,有轻微的异型性。未见鳞状上皮颗粒层和角化(图2,a),而瘤巢中可见明显的粘蛋白沉积(图2,B)。此外,囊肿壁部分变薄(图2,C)。免疫组化检查,瘤巢上皮膜抗原(EMA)(图2,D)、癌胚抗原和囊性疾病液体蛋白15均阴性。阿利新蓝染色间质黏液阳性(图2,E),诊断为囊性基底细胞癌。术后20个月未见复发迹象。形成单个囊肿的BCC,特别是完全由BCC细胞组成或由表皮样囊肿发展而来的BCC非常罕见;然而,在少数病例中,囊肿壁由带有角透明素颗粒的鳞状细胞组成,肿瘤的某些部位存在BCC细胞(3,4)。在这些病例中,肿瘤的大小是
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引用次数: 0
The Influence of Psychological Stress on the Levels of the Skin's Natural Moisturizing Factor in Croatian Medical Students. 心理压力对克罗地亚医学生皮肤天然保湿因子水平的影响
Mislav Mokos, Ivana Orešković, Dora Vlašić, Maja Mikulec, Ivone Jakaša, Branka Marinović

Psychological stress may affect skin barrier homeostasis and slow down its recovery. Therefore, we aimed to examine the association between psychological stress levels and natural moisturizing factor (NMF) levels in the stratum corneum (SC). NMF levels were determined in the SC of 25 third-year and 25 sixth-year medical students in two periods (stressful and non-stressful) using the tape stripping method, high-performance liquid chromatography, and spectrophotometry. Additionally, students completed the Perceived Stress Scale questionnaire. Most students had medium stress levels. During the non-stressful period, third-year students were under higher stress than sixth-year students (P<0.001). However, there was no significant difference between the two examined groups in the stressful period. Only among the sixth-year students were significantly higher stress levels found in the stressful period compared with the non-stressful period. In both groups, the levels of NMF did not differ significantly between the examined periods. Regarding the non-stressful period, there was a clear trend of increasing NMF levels among third-year students compared with sixth-year students (P=0.0547), consistent with the higher stress levels among third-year students during this period. Additionally, sixth-year female students had significantly lower NMF levels during the non-stressful period than third-year female students. In conclusion, third-year students were exposed to longer-term stress, while the stress of sixth-year students was associated with the exam-writing period. Additionally, we observed a trend of increasing NMF levels with increasing stress. Further research is needed to determine the mechanism by which stress, as an independent factor, influences NMF levels in the SC.

心理压力会影响皮肤屏障的平衡,减缓其恢复。因此,我们旨在研究角质层(SC)中心理应激水平与自然保湿因子(NMF)水平之间的关系。采用胶带剥离法、高效液相色谱法和分光光度法测定了25名三年级和六年级医学生应激期和非应激期SC中的NMF水平。此外,学生还完成了压力感知量表问卷。大多数学生的压力处于中等水平。在非压力期,三年级学生的压力高于六年级学生(P
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引用次数: 0
Cutaneous Leishmaniasis Masquerading as Diabetic Foot: A Call for Vigilance. 伪装成糖尿病足的皮肤利什曼病:提高警惕。
Khalid Al Aboud, Ahmad Al Aboud

parts of the world (1,2). CL is characterized by significant clinical variability. An ulcerated nodule on the exposed parts of the body (corresponding to the parasite inoculation site by the vector insect) is the classic presentation. However, other forms of clinical presentations also exist (3,4). CL can be present on unusual locations such as the scalp, the genital region, or palmoplantar areas. Localization in the foot poses a diagnostic challenge due to similarity to disorders which produce ulcerations. The latter include diabetes and leprosy. A 75-year-old Afghani man presented with a large, progressive, cutaneous necrotic ulcer on the left foot (Figure 1) associated with pain, one year in duration. The patient has no history of trauma or any chronic systemic disorders. Additionally, the patient reported no similar diseases in the past. The patient was repeatedly diagnosed with diabetic foot in his village. Dressings were administered several times without any improvement. The patient underwent blood tests, including fasting blood sugar, which were all within normal limits. As the patient lived in an endemic area for CL, CL was suspected and confirmed by slit skin smear and skin biopsy. The latter demonstrated Leishmania amastigotes in the dermal histiocytic infiltrates (Figure 2). The patient is currently under treatment with systemic sodium stibogluconate (pentostam). Leishmaniasis is a major medical issue in several parts of the world. It is transmitted by sandflies. Visceral and cutaneous forms of the disease have been identified. There are more than 1.5 million cases of CL reported annually around the world (1-4). The classical form of CL can be easily diagnosed, particularly in the endemic areas. However, rare and an unusual clinical locations and presentations exist. The latter include annular, chancriform, acute paronychial, palmoplantar, zosteriform, and erysipeloid. Involvement of lower limbs is not common, but can occur (1,2). CL affecting the foot is much less commonly reported (3,4). In this location, CL can be confused with other conditions such as leprosy, vasculitis, neoplastic ulcers, and chronic ulcers due to vascular insufficiency or diabetes. Some authors have stated that routine diagnostic biopsies may be useful in case of clinically suspected wound infections, particularly in patients with deep ulcerations, diabetic foot syndrome, severe soft tissue infection, or fistula tissue. They believed that biopsies are indispensable in the microbiology workup of specific pathogens such as mycobacteria, Leishmania, actinomycetes, Nocardia ssp., or molds (5). The present case highlights the importance of proper investigation of foot ulcer and the importance of considering the diagnosis of leishmaniasis, particularly in endemic areas.

1,2)。CL的临床表现差异很大。典型的表现是身体暴露部位出现溃疡性结节(与病媒昆虫的寄生虫接种部位相对应)。不过,也有其他形式的临床表现(3,4)。CL 可出现在异常部位,如头皮、生殖器或掌跖部位。由于与产生溃疡的疾病相似,足部局部病变给诊断带来了挑战。后者包括糖尿病和麻风病。一名 75 岁的阿富汗籍男子左脚出现大面积、进行性、皮肤坏死性溃疡(图 1),伴有疼痛,病程一年。患者没有外伤史或任何慢性全身性疾病。此外,患者称过去没有类似疾病。患者在村里多次被诊断为糖尿病足。曾多次进行敷料治疗,但病情未见好转。患者接受了包括空腹血糖在内的血液检查,结果均在正常范围内。由于患者生活在利什曼病流行区,因此怀疑他患有利什曼病,并通过裂隙皮肤涂片和皮肤活检进行了确诊。活检结果显示,真皮组织细胞浸润中存在利什曼原虫(图 2)。患者目前正在接受全身性斯地巴葡萄糖酸钠(喷妥斯汀)治疗。利什曼病是世界多个地区的主要医学问题。它由沙蝇传播。已发现有内脏型和皮肤型利什曼病。全世界每年报告的利什曼病病例超过 150 万例(1-4)。传统形式的 CL 很容易诊断,尤其是在流行地区。然而,也有罕见和不寻常的临床部位和表现。后者包括环状型、疳积型、急性腮腺炎型、掌跖型、带状疱疹型和红斑狼疮型。下肢受累并不常见,但也可能发生(1,2)。CL累及足部的报道较少(3,4)。在这种情况下,CL 可能会与其他疾病混淆,如麻风病、血管炎、肿瘤性溃疡以及血管功能不全或糖尿病引起的慢性溃疡。一些作者指出,在临床上怀疑伤口感染的情况下,常规诊断性活检可能会有用,尤其是在深部溃疡、糖尿病足综合征、严重软组织感染或瘘管组织的患者中。他们认为,活检对于特定病原体(如分枝杆菌、利什曼原虫、放线菌、诺卡氏菌或霉菌)的微生物学检查是不可或缺的(5)。本病例强调了对足部溃疡进行适当检查的重要性,以及考虑利什曼病诊断的重要性,尤其是在利什曼病流行地区。
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引用次数: 0
Safety and Efficacy of Long-term Use of Infliximab in Severe Juvenile Dermatomyositis - 12 Years of Follow-up. 长期使用英夫利西单抗治疗严重幼年皮肌炎的安全性和有效性--12 年随访。
Nastasia Kifer, Marijan Frković, Sanja Perić, Mario Šestan, Nenad Vukojević, Marija Jelušić

Juvenile dermatomyositis with emphasized vasculopathy is rare, but the most severe form of the disease, with a poor prognosis with relapsing and chronic course or, in some cases, lethal outcome. We present a case of a 19-year-old Caucasian female, who developed severe acute juvenile dermatomyositis with emphasized multisystem vasculopathy, including retinal vasculopathy and maculopathy (cotton-wool spots, retinal hemorrhages, macular edema) at the age of 8. Due to no response to standard treatment protocols and rapid worsening of clinical symptoms and laboratory findings, a TNF inhibitor (infliximab) was introduced after the third week of treatment resulting in complete normalisation of muscle enzyme levels and complete resolution of eye changes within the next 2 weeks with a gradual general recovery. To the best of our knowledge, this is the first long-term follow-up of an early TNF inhibitor introduction in a patient with acute, severe form of juvenile dermatomyositis and retinal vasculopathy. After 12 years of infliximab therapy, the outcome was excellent, with no side effects throughout the whole treatment.

青少年皮肌炎伴血管病变是罕见的,但却是该疾病最严重的形式,预后差,可复发和慢性病程,在某些情况下,可导致致命的结果。我们报告一个19岁的白人女性病例,她在8岁时发展为严重的急性青少年皮肌炎,并伴有多系统血管病变,包括视网膜血管病变和黄斑病变(棉斑、视网膜出血、黄斑水肿)。由于对标准治疗方案无反应,临床症状和实验室检查结果迅速恶化,在治疗第三周后引入TNF抑制剂(英夫利昔单抗),导致肌肉酶水平完全正常化,并在接下来的2周内完全消除眼部变化,逐渐全面恢复。据我们所知,这是第一个长期随访的早期TNF抑制剂引入急性,严重形式的青少年皮肌炎和视网膜血管病变的患者。经过12年的英夫利昔单抗治疗,结果非常好,在整个治疗过程中没有副作用。
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引用次数: 0
Phototoxic reaction to oral terbinafine due to Tinea capitis in a child. 一名儿童因患头癣而口服特比萘芬后出现光毒性反应。
Ana Bakija-Konsuo, Lena Kotrulja, Matko Marlais
<p><p>We report the case of an 18-month-old boy who developed a phototoxic skin reaction to terbinafine on his scalp, ears, and face in the form of disseminated erythematous plaques, which resembled subacute lupus erythematosus (SCLE) in their clinical presentation. Skin changes appeared a short time after the boy was exposed to sunlight during the period of time when he was treated with oral terbinafine due to Microsporum canis fungal scalp infection. Tinea capitis is a common dermatophyte infection primarily affecting prepubertal children (1). Microsporum canis remains the predominant causative organism in many countries of the Mediterranean basin, the most important dermatophyte carriers being stray cats and dogs. Systemic therapy is required for treatment because topical antifungal agents do not penetrate down to the deepest part of the hair follicle (2). Terbinafine is commonly used in the treatment of microsporosis, as its fungicidal action permits short periods of treatment (3,4). The first skin changes occurred in the parietal scalp region in the form of round scaly alopecia, with the presence of unevenly broken hairs and enlarged regional lymph nodes (Figure 1). Diagnosis of fungal infection included clinical assessment and Wood's light examination, which revealed green-yellow fluorescence on the lesional scalp region. Fungal culture identification was performed according to conventional methods, revealing fungal culture positive for dermatophytes from the genus Microsporum canis. The boy had a history of contact with a cat. Systemic therapy with the oral antifungal drug terbinafine was administered at a dose of 62.5 mg per day (5 mg/kg), with topical application of antifungal cream (miconazole), 10% Ichthyol cream in the evening, and antifungal shampoo (ketoconazole) twice a week. After two weeks of therapy, we observed initial regression of scalp lesions. Oral terbinafine was well-tolerated, and the patient did not experience any side-effects. Laboratory findings included liver function tests and were within normal ranges. At this point, the oral dose of terbinafine was increased to 125 mg per day (10 mg/kg) at a revised schedule according to body weight: 10-25 kg, 125 mg/day (5). Approximately five weeks after starting the treatment with oral terbinafine, after the boy was exposed to the sun, acute disseminated erythematosus lesions appeared on the face and scalp. Clinical presentation of the lesions and acute onset during exposure to sunlight raised the suspicion of a phototoxic reaction to terbinafine (Figure 2). The patient was not taking any other medication at that time, had no history of drug or food allergies, and had not previously experienced photosensitive skin reactions. Due to the inflamed skin changes resembling subacute lupus and photosensitivity, an immunological assay tests were also performed. Due to the young age of the patient, no skin biopsy or photo-patch test was performed. Despite the recent skin changes and sus
我们报告了一例18个月大的男孩,他的头皮、耳朵和面部出现了特比萘芬的光毒性皮肤反应,表现为弥散性红斑斑块,其临床表现与亚急性红斑狼疮(SCLE)相似。由于犬小孢子菌头皮真菌感染,在口服特比萘芬治疗期间,男孩在阳光照射后短时间内出现皮肤变化。头癣是一种常见的皮肤真菌感染,主要影响青春期前的儿童(1)。在地中海盆地的许多国家,犬小孢子虫仍然是主要的致病生物,最重要的皮肤真菌携带者是流浪猫和狗。治疗需要全身治疗,因为局部抗真菌药物不能渗透到毛囊的最深处(2)。特比萘芬通常用于治疗小孢子病,因为它的杀真菌作用允许短期治疗(3,4)。最初的皮肤变化发生在头皮顶区,表现为圆形鳞状脱发,伴有不均匀的毛发折断和区域淋巴结肿大(图1)。真菌感染的诊断包括临床评估和Wood's光检查,病变头皮区域显示黄绿色荧光。按照常规方法进行真菌培养鉴定,结果显示犬小孢子菌属皮肤真菌培养阳性。这名男孩曾接触过猫。口服抗真菌药物特比萘芬进行全身治疗,剂量为每天62.5 mg (5mg /kg),局部使用抗真菌乳膏(咪康唑),晚上使用10%鱼油乳膏,每周两次使用抗真菌洗发水(酮康唑)。治疗两周后,我们观察到头皮病变的初步消退。口服特比萘芬耐受性良好,患者未出现任何副作用。实验室检查结果包括肝功能检查,在正常范围内。此时,口服特比萘芬剂量根据体重增加到125mg /天(10mg /kg): 10- 25kg, 125mg /天(5)。口服特比萘芬治疗开始约五周后,男孩暴露在阳光下后,面部和头皮出现急性弥散性红斑病变。病变的临床表现和暴露在阳光下的急性发作引起了对特比萘芬光毒性反应的怀疑(图2)。患者当时没有服用任何其他药物,没有药物或食物过敏史,以前也没有发生过光敏性皮肤反应。由于发炎的皮肤变化类似亚急性狼疮和光敏性,免疫分析测试也进行了。由于患者年龄小,未进行皮肤活检或光贴试验。尽管最近皮肤发生变化并怀疑药物引起的光毒性,但由于真菌学结果持续阳性(Wood光和真菌培养),口服特比萘芬继续治疗。父母被建议严格防止孩子暴露在阳光下。治疗三个月后,一旦获得第二次阴性真菌培养并实现病变的临床消退,就完成了特比萘芬的全身治疗。急性播散性炎症性光毒性皮肤病变,局部应用皮质类固醇霜(糠酸莫他酮),严格避免阳光照射,并使用防晒霜。炎症性皮肤变化在使用皮质类固醇软膏治疗后几天内完全消退。免疫检测结果均为完全阴性(ANA、CIC-IgG、C3、抗ds- dna、抗ro /SS-A、抗la /SS-B、抗sm1、抗u1rnp、抗组蛋白抗体、抗PmScl、抗PCNA)。当面对阳光照射后出现的皮疹时,外源性光敏化是最可能的原因,特别是在引入特比萘芬后突然出现临床症状,并且以前没有太阳过敏史。光敏性药物疹,包括光毒性和光过敏,已经报道了许多全身药物。特比萘芬引起的光敏性似乎非常罕见。到目前为止,只有特比萘芬引起的红斑狼疮(LE)、亚急性皮肤LE或特比萘芬加重的LE的孤立病例被报道(6)。作为特比萘芬的副作用,光毒性反应很少被报道(例如,光性皮炎、过敏性光敏和多形光疹)(7)。在文献中,我们只发现1例患者对口服特比萘芬有光过敏反应。作者通过照片贴片测试证实了这一点(8)。 已有特比萘芬引起的亚急性皮肤红斑狼疮(SCLE)和系统性红斑狼疮因特比萘芬而加重的病例报道,免疫检测阳性,抗组蛋白抗体阳性(9)。对该患者的进一步监测显示,炎症性皮肤变化未复发,而由于深度真菌性皮肤感染,头皮上仍有两个小的残留脱发疤痕。在患者接受特比萘芬治疗时,暴露在阳光下皮肤出现炎症变化,局部皮质类固醇治疗后皮肤炎症迅速消退,免疫测定结果阴性,这使得特比萘芬的光毒性反应的诊断得以确立。特比萘芬在文献中很少被提及具有光敏性,但这个临床病例提供了一个例子,并强调了关于这种副作用的药物建议的重要性,特别是在夏季(7)。
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引用次数: 0
A Pediatric Case of Multiple Bilateral Symmetric Eccrine Angiomatous Hamartoma. 多发性双侧对称性肾小球血管瘤的儿科病例。
Ilke Beyitler, Fikret Dirilenoglu

Eccrine angiomatous hamartoma (EAH) is a rare benign skin neoplasm characterized by an increased size and number of eccrine glands or ducts, along with proliferation of vascular structures in the dermis. This case is unique in its presentation of bilateral symmetrical nodules on both hands and the development of new nodules during puberty. It highlights the need for further research and understanding of this rare condition and its potential progression over time.

内分泌血管瘤错构瘤(EAH)是一种罕见的良性皮肤肿瘤,其特征是内分泌腺体或导管的大小和数量增加,并伴有真皮血管结构的增生。这种情况是独特的,其表现为双侧对称结节在双手和发展的新结节在青春期。它强调了进一步研究和了解这种罕见疾病及其潜在进展的必要性。
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引用次数: 0
期刊
Acta dermatovenerologica Croatica : ADC
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