一名达里尔病患者的卡波西静脉曲张溃疡:罕见病例报告

Shirahatti Trishala, H. Bangaru
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摘要

达里尔病是一种罕见的常染色体显性遗传疾病,临床特征是在脂溢区出现多发性、瘙痒性、离散性和鳞屑性丘疹,同时伴有手掌凹陷、指甲变化和粘膜受累。组织学上,病变表现为基底上裂隙,伴有棘层细胞溶解和角化不良细胞。卡波西静脉曲张样疹子(KVE)是单纯疱疹病毒在原有皮肤病的基础上的传播,表现为播散性水疱。达里尔病表现为 KVE 的情况非常罕见。如果误诊或不及时治疗,会增加死亡率。在此,我们报告了一例 25 岁男性患者的病例,他的面部、颈部、胸部和上背部出现多发性水泡和结痂性病变。经过全面的皮肤检查,发现他自童年起就在脂溢部位出现多发性角化过度、脏疣状丘疹。经过详细的临床和实验室评估,我们诊断他患有KVE,并伴有潜在的达里尔病。
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Kaposi’s Varicelliform Eruption in a Patient with Darier’s Disease: A Rare Case Report
Darier's disease is a rare autosomal dominant disorder, characterized clinically by the appearance of multiple, pruritic, discrete, and scaly papules affecting seborrheic areas coupled with palmar pits, nail changes, and mucosal involvement. Histologically, the lesions show supra-basal clefts with acantholytic and dyskeratotic cells. Kaposi's varicelliform eruption (KVE) is the dissemination of the herpes simplex virus in the setting of preexisting skin disease, presenting with disseminated vesico-pustules. Darier's disease presenting as KVE is a rare occurrence. If misdiagnosed or left untreated can increase the rate of mortality. Here, we report the case of a 25-year-old male patient who presented with multiple vesicles and crusted lesions over the face, neck, chest, and upper back. After thorough cutaneous examination, he was found to have multiple hyperkeratotic, dirty-warty papules over seborrheic areas, present since childhood. With detailed clinical and laboratory evaluation, we arrived at the diagnosis of KVE with underlying Darier disease.
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