Familial Reactive Perforating Collagenosis with Adolescence-Onset: A Rare Case Report and Literature Review.

IF 1.9 4区 医学 Q3 DERMATOLOGY
Clinical, Cosmetic and Investigational Dermatology Pub Date : 2024-08-23 eCollection Date: 2024-01-01 DOI:10.2147/CCID.S468181
Laila Tsaqilah, Hedwika Advina Nastiti, Hermin Aminah Usman, Erda Avriyanti, Hartati Purbo Dharmadji, Risa Miliawati Nurul Hidayah
{"title":"Familial Reactive Perforating Collagenosis with Adolescence-Onset: A Rare Case Report and Literature Review.","authors":"Laila Tsaqilah, Hedwika Advina Nastiti, Hermin Aminah Usman, Erda Avriyanti, Hartati Purbo Dharmadji, Risa Miliawati Nurul Hidayah","doi":"10.2147/CCID.S468181","DOIUrl":null,"url":null,"abstract":"<p><p>Familial Reactive Perforating Collagenosis (FRPC) is a very rare form of benign dermatosis frequently presented during early childhood and not associated with systemic diseases. Less than 50 FRPC patients have been reported in the literature. Due to the limited number of cases, the pathophysiology of this unique entity remains elusive; moreover, no standard treatment has been agreed upon. Here, we report a case of FRPC in a 20-year-old male who was presented with generalized multiple discrete papules covered with central keratotic plugs in all regions of his body, particularly in the facial area, neck, abdominal, and extensor region of the extremities for more than 7 years. Similar symptoms were acknowledged in the patient's family members. Histopathological analyses identified the crateriform shape invagination in the epidermis filled with inflammatory lymphocytes and basophilic debris and perforated by basophilic collagen bundles from the underlying dermis. Based on the clinical and histopathological findings, the patient was diagnosed with FRPC. He was treated with topical desoximetasone 0.25% cream applied 2-3 times daily. A follow-up evaluation after 4 weeks revealed a near-complete resolution of skin papules. To our knowledge, this is the first report of FRPC case from Indonesia. Unlike the majority of FRPC patients who had their disease onsets during infancy or early childhood, FRPC skin manifestations in our patient started during the adolescence period. The resolution of skin manifestations after daily application of topical desoximetasone suggests that topical corticosteroids are a potential treatment option for FRPC patients.</p>","PeriodicalId":10447,"journal":{"name":"Clinical, Cosmetic and Investigational Dermatology","volume":null,"pages":null},"PeriodicalIF":1.9000,"publicationDate":"2024-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11348990/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Clinical, Cosmetic and Investigational Dermatology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.2147/CCID.S468181","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/1/1 0:00:00","PubModel":"eCollection","JCR":"Q3","JCRName":"DERMATOLOGY","Score":null,"Total":0}
引用次数: 0

Abstract

Familial Reactive Perforating Collagenosis (FRPC) is a very rare form of benign dermatosis frequently presented during early childhood and not associated with systemic diseases. Less than 50 FRPC patients have been reported in the literature. Due to the limited number of cases, the pathophysiology of this unique entity remains elusive; moreover, no standard treatment has been agreed upon. Here, we report a case of FRPC in a 20-year-old male who was presented with generalized multiple discrete papules covered with central keratotic plugs in all regions of his body, particularly in the facial area, neck, abdominal, and extensor region of the extremities for more than 7 years. Similar symptoms were acknowledged in the patient's family members. Histopathological analyses identified the crateriform shape invagination in the epidermis filled with inflammatory lymphocytes and basophilic debris and perforated by basophilic collagen bundles from the underlying dermis. Based on the clinical and histopathological findings, the patient was diagnosed with FRPC. He was treated with topical desoximetasone 0.25% cream applied 2-3 times daily. A follow-up evaluation after 4 weeks revealed a near-complete resolution of skin papules. To our knowledge, this is the first report of FRPC case from Indonesia. Unlike the majority of FRPC patients who had their disease onsets during infancy or early childhood, FRPC skin manifestations in our patient started during the adolescence period. The resolution of skin manifestations after daily application of topical desoximetasone suggests that topical corticosteroids are a potential treatment option for FRPC patients.

青春期发病的家族性反应性穿孔性胶原病:罕见病例报告和文献综述。
家族性反应性胶原穿孔病(FRPC)是一种非常罕见的良性皮肤病,常在儿童早期发病,与全身性疾病无关。文献中报道的家族性反应性胶原穿孔症患者不足 50 例。由于病例数量有限,这种独特病症的病理生理学仍然难以捉摸;此外,还没有达成一致的标准治疗方法。在此,我们报告了一例 20 岁男性 FRPC 患者的病例,该患者全身各处,尤其是面部、颈部、腹部和四肢伸侧,出现全身多发离散性丘疹,表面覆盖中央角化栓,病程超过 7 年。患者的家人也有类似症状。组织病理学分析发现,表皮中的火山口状内陷充满了炎性淋巴细胞和嗜碱性碎屑,真皮下层的嗜碱性胶原束穿孔。根据临床和组织病理学检查结果,患者被诊断为 FRPC。他接受了 0.25% 去羟米松乳膏的局部治疗,每天涂抹 2-3 次。4 周后的随访评估显示,皮肤丘疹几乎完全消退。据我们所知,这是印度尼西亚首次报告 FRPC 病例。与大多数在婴儿期或幼儿期发病的 FRPC 患者不同,本例患者的 FRPC 皮肤表现始于青春期。每天外用去羟米松(desoximetasone)后,皮肤表现有所缓解,这表明外用皮质类固醇激素是治疗 FRPC 患者的一种潜在方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 求助全文
来源期刊
CiteScore
2.80
自引率
4.30%
发文量
353
审稿时长
16 weeks
期刊介绍: Clinical, Cosmetic and Investigational Dermatology is an international, peer-reviewed, open access journal that focuses on the latest clinical and experimental research in all aspects of skin disease and cosmetic interventions. Normal and pathological processes in skin development and aging, their modification and treatment, as well as basic research into histology of dermal and dermal structures that provide clinical insights and potential treatment options are key topics for the journal. Patient satisfaction, preference, quality of life, compliance, persistence and their role in developing new management options to optimize outcomes for target conditions constitute major areas of interest. The journal is characterized by the rapid reporting of clinical studies, reviews and original research in skin research and skin care. All areas of dermatology will be covered; contributions will be welcomed from all clinicians and basic science researchers globally.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信