O. Chernysheva, O. Dorokhina, A. Khlebnikova, E. Selezneva
{"title":"成人起病的朗格汉斯细胞组织细胞增多症:一例临床病例","authors":"O. Chernysheva, O. Dorokhina, A. Khlebnikova, E. Selezneva","doi":"10.18786/2072-0505-2021-49-064","DOIUrl":null,"url":null,"abstract":"Langerhans cell histiocytosis is mainly diagnosed in children, and its manifestation in adult age is quite uncommon. Skin rashes may be non-specific and mimic a number of dermatoses. Therefore, the clinical diagnosis is challenging and as a rule, such patients are misinterpreted and managed for other disorders by a dermatologist for some years. \nWe present a clinical case of Langerhans cell histiocytosis with skin involvement in a 35-year female patient, who had been treated by a dermatologist for 2 years for pyoderma, seborrheic dermatitis, and skin fold candidiasis. Taking into account the clinical signs and symptoms and age of manifestation, we initially suspected familial benign pemphigus (Hailey-Hailey disease) or follicular dyskeratosis (Darier's disease). However, the histological assessment showed Langerhans cell histiocytosis confirmed by immunohistochemistry with anti-langerin, anti-CD1a, and anti-protein S-100 antibodies. The patient was referred to a hematologist for further work-upand specific treatment. \nIn cases of any treatment resistant disorders, which do not respond to long-term conventional treatment, it is necessary to reconsider the diagnosis by means of histological investigation. It would allow for identification of a disease, which is uncommon in dermatology practice.","PeriodicalId":7638,"journal":{"name":"Almanac of Clinical Medicine","volume":"1 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2022-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"1","resultStr":"{\"title\":\"Adult-onset of Langerhans cell histiocytosis: a clinical case\",\"authors\":\"O. Chernysheva, O. Dorokhina, A. Khlebnikova, E. Selezneva\",\"doi\":\"10.18786/2072-0505-2021-49-064\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Langerhans cell histiocytosis is mainly diagnosed in children, and its manifestation in adult age is quite uncommon. Skin rashes may be non-specific and mimic a number of dermatoses. Therefore, the clinical diagnosis is challenging and as a rule, such patients are misinterpreted and managed for other disorders by a dermatologist for some years. \\nWe present a clinical case of Langerhans cell histiocytosis with skin involvement in a 35-year female patient, who had been treated by a dermatologist for 2 years for pyoderma, seborrheic dermatitis, and skin fold candidiasis. Taking into account the clinical signs and symptoms and age of manifestation, we initially suspected familial benign pemphigus (Hailey-Hailey disease) or follicular dyskeratosis (Darier's disease). However, the histological assessment showed Langerhans cell histiocytosis confirmed by immunohistochemistry with anti-langerin, anti-CD1a, and anti-protein S-100 antibodies. The patient was referred to a hematologist for further work-upand specific treatment. \\nIn cases of any treatment resistant disorders, which do not respond to long-term conventional treatment, it is necessary to reconsider the diagnosis by means of histological investigation. It would allow for identification of a disease, which is uncommon in dermatology practice.\",\"PeriodicalId\":7638,\"journal\":{\"name\":\"Almanac of Clinical Medicine\",\"volume\":\"1 1\",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2022-01-21\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"1\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Almanac of Clinical Medicine\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.18786/2072-0505-2021-49-064\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Almanac of Clinical Medicine","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.18786/2072-0505-2021-49-064","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Adult-onset of Langerhans cell histiocytosis: a clinical case
Langerhans cell histiocytosis is mainly diagnosed in children, and its manifestation in adult age is quite uncommon. Skin rashes may be non-specific and mimic a number of dermatoses. Therefore, the clinical diagnosis is challenging and as a rule, such patients are misinterpreted and managed for other disorders by a dermatologist for some years.
We present a clinical case of Langerhans cell histiocytosis with skin involvement in a 35-year female patient, who had been treated by a dermatologist for 2 years for pyoderma, seborrheic dermatitis, and skin fold candidiasis. Taking into account the clinical signs and symptoms and age of manifestation, we initially suspected familial benign pemphigus (Hailey-Hailey disease) or follicular dyskeratosis (Darier's disease). However, the histological assessment showed Langerhans cell histiocytosis confirmed by immunohistochemistry with anti-langerin, anti-CD1a, and anti-protein S-100 antibodies. The patient was referred to a hematologist for further work-upand specific treatment.
In cases of any treatment resistant disorders, which do not respond to long-term conventional treatment, it is necessary to reconsider the diagnosis by means of histological investigation. It would allow for identification of a disease, which is uncommon in dermatology practice.