{"title":"Pigmenti 失禁症:六例孤立皮肤受累的系列病例。","authors":"Bhumesh K Katakam, Narsimha Rao Netha Gurram, Sudharani Chintagunta, Arunima Dhabal","doi":"10.4103/idoj.idoj_447_23","DOIUrl":null,"url":null,"abstract":"<p><p>Incontinentia pigmenti (IP) is a rare multi-system genetic disorder mostly affecting females. It presents primarily with cutaneous lesions but is often associated with dental, ocular, neurological, musculoskeletal, and cardiovascular abnormalities. We report a series of one male and five female infants with IP having isolated cutaneous involvement at the time of presentation. In such cases, timely diagnosis of the condition, followed by systemic evaluation and long-term periodic follow-up, is imperative to detect and treat more serious systemic manifestations at an early stage.</p>","PeriodicalId":13335,"journal":{"name":"Indian Dermatology Online Journal","volume":"15 2","pages":"259-262"},"PeriodicalIF":1.9000,"publicationDate":"2024-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10969251/pdf/","citationCount":"0","resultStr":"{\"title\":\"Incontinentia Pigmenti: A Series of Six Cases with Isolated Cutaneous Involvement.\",\"authors\":\"Bhumesh K Katakam, Narsimha Rao Netha Gurram, Sudharani Chintagunta, Arunima Dhabal\",\"doi\":\"10.4103/idoj.idoj_447_23\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Incontinentia pigmenti (IP) is a rare multi-system genetic disorder mostly affecting females. It presents primarily with cutaneous lesions but is often associated with dental, ocular, neurological, musculoskeletal, and cardiovascular abnormalities. We report a series of one male and five female infants with IP having isolated cutaneous involvement at the time of presentation. In such cases, timely diagnosis of the condition, followed by systemic evaluation and long-term periodic follow-up, is imperative to detect and treat more serious systemic manifestations at an early stage.</p>\",\"PeriodicalId\":13335,\"journal\":{\"name\":\"Indian Dermatology Online Journal\",\"volume\":\"15 2\",\"pages\":\"259-262\"},\"PeriodicalIF\":1.9000,\"publicationDate\":\"2024-02-13\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10969251/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Indian Dermatology Online Journal\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4103/idoj.idoj_447_23\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/3/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q3\",\"JCRName\":\"DERMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Indian Dermatology Online Journal","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/idoj.idoj_447_23","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/3/1 0:00:00","PubModel":"eCollection","JCR":"Q3","JCRName":"DERMATOLOGY","Score":null,"Total":0}
引用次数: 0
摘要
猪软化症(IP)是一种罕见的多系统遗传性疾病,女性患者居多。它主要表现为皮肤病变,但经常伴有牙齿、眼部、神经、肌肉骨骼和心血管异常。我们报告了一系列患 IP 的婴儿病例,其中一名男婴和五名女婴在发病时仅有皮肤受累。在这种情况下,必须及时诊断病情,进行系统评估和长期定期随访,以便及早发现和治疗更严重的全身表现。
Incontinentia Pigmenti: A Series of Six Cases with Isolated Cutaneous Involvement.
Incontinentia pigmenti (IP) is a rare multi-system genetic disorder mostly affecting females. It presents primarily with cutaneous lesions but is often associated with dental, ocular, neurological, musculoskeletal, and cardiovascular abnormalities. We report a series of one male and five female infants with IP having isolated cutaneous involvement at the time of presentation. In such cases, timely diagnosis of the condition, followed by systemic evaluation and long-term periodic follow-up, is imperative to detect and treat more serious systemic manifestations at an early stage.