Epidermodysplasia Verruciformis (Lewandowsky and Lutz's Dysplasia).

Skinmed Pub Date : 2024-09-17 eCollection Date: 2024-01-01
Avita Dhiman, Naveen Kumar Kansal, Pakesh Baishya
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Abstract

A woman in her twenties, with a non-consanguineous marriage, presented to the dermatology clinic with asymptomatic lesions on her face, neck, trunk, and extremities for the past 12 years. The general physical and systemic examination was unremarkable. Cutaneous examination revealed multiple hypopigmented to a few hyperpigmented, slightly scaly tinea versicolor-like macules distributed predominantly on the neck, upper portion of the back, and distal parts of the extremities (Figures 1-5). There were muultiple, slightly erythematous to violaceous flat-topped wart-like papules and plaques were discovered on the arms (Figures 1 and 2), with seborrheic keratosis-like lesions on the face (Figure 5). Baseline investigations, such as complete blood count (CBC), liver function test (LFT), kidney function test (KFT), and plasma glucose levels, were normal. Serologic tests conducted for human immunodeficiency virus (HIV)-1 and HIV-2, and hepatitis B and C were nonreactive. Skin lesion potassium hydroxide (KOH) examination from tinea versicolor-like lesions was negative for fungal elements. Skin biopsies were performed from tinea versicolor-like macules present on the upper portion of the back, flat wart-like plaque on the dorsum of the left hand, and hyperpigmented scaly plaque in the extensor area of the left leg. Histopathologic examination revealed parakeratosis, hyperkeratosis, and acanthosis with swollen keratinocytes, bluish-gray cytoplasm, and rounded nuclei with prominent nucleoli. No dysplastic changes/atypia or mitotic figures (Figures 6 and 7), plus occasional perinuclear halo (a vacuolated area that surrounds the nucleus), were observed. Following clinicopathologic correlation, the patient was diagnosed with epidermodysplasia verruciformis (EV, or Lewandowsky and Lutz's dysplasia).

疣状表皮增生症(Lewandowsky 和 Lutz's疣)。
一名 20 多岁的女性患者因面部、颈部、躯干和四肢在过去 12 年中出现无症状皮损而到皮肤科就诊,她的婚姻并非近亲结婚。一般体格检查和全身检查均无异常。皮肤检查发现多处色素减退至少数色素沉着、略带鳞屑的天疱疮样斑疹,主要分布在颈部、背部上半部分和四肢远端(图 1-5)。在手臂上发现了多个略带红斑至粟米色的平顶疣状丘疹和斑块(图 1 和图 2),面部有脂溢性角化病样皮损(图 5)。全血细胞计数(CBC)、肝功能检测(LFT)、肾功能检测(KFT)和血浆葡萄糖水平等基线检查均正常。人体免疫缺陷病毒(HIV)-1、HIV-2、乙型肝炎和丙型肝炎血清学检测均无反应。对类似天疱疮的皮损进行氢氧化钾(KOH)检查,结果显示真菌成分呈阴性。对背部上半部分的天疱疮样斑疹、左手背上的扁平疣状斑块和左腿伸展部位的色素沉着性鳞屑斑块进行了皮肤活检。组织病理学检查显示,患者患有角化不全、角化过度和棘皮症,角质细胞肿胀,细胞质呈蓝灰色,细胞核呈圆形,核仁突出。未见增生异常改变/畸形或有丝分裂图形(图 6 和图 7),偶尔可见核周晕(细胞核周围的空泡区)。经临床病理学相关分析,患者被诊断为表皮增生性疣状增生(EV,或 Lewandowsky 和 Lutz 发育不良)。
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