Ivana Ilijin, Z. Mijuskovic, D. Brasanac, L. Kandolf Sekulovič
{"title":"嗜滤泡性蕈样真菌病1例报告","authors":"Ivana Ilijin, Z. Mijuskovic, D. Brasanac, L. Kandolf Sekulovič","doi":"10.1515/sjdv-2016-0019","DOIUrl":null,"url":null,"abstract":"Abstract Mycosis fungoides (MF) belongs to a group of primary cutaneous T-cell lymphomas, with characteristic small- to medium-sized neoplastic T-lymphocytes with hyperchromatic and cerebriform nuclei. Folliculotropic mycosis fungoides (FMF) represents a variant of mycosis fungoides, which is histologically characterized by folliculotropic T-cell infiltrates, with or without mucinous degeneration of the hair follicles. Clinical features of FMF are characterized by appearance of grouped follicular papules, acneiform lesions, indurated plaques, sometimes tumors, which usually involve the head and neck region. The diagnosis is based on clinical presentation, histopathological and immunohistochemical (IHC) findings of skin biopsy specimens. The treatment of FMF, and generally MF, should be stage-adapted. Case report: We present a case of a 33-year-old male with an eight-month history of erythematous papules on his forehead accompanied by intense pruritus. Histopathological findings showed folliculotropic and perivascular lymphocytic infiltrates. An increased CD4/CD8 ratio of interfollicular lymphocytes with accumulation of Langerhans cell confirmed the diagnosis of FMF. Our patient was diagnosed with an early stage - IA, and P-UVA phototherapy was recommended due to ineffectiveness of prescribed topical corticosteroids that had shortterm effects. Conclusion: Folliculotropic mycosis fungoides represents a diagnostic challenge due to the great diversity of clinical manifestations. We presented a rare case of folliculotropic mycosis fungoides in a young adult, who presented with erythematous papules, accompanied by intense pruritus on the forehead, which lasted for several months. Histopathological and IHC analysis confirmed the diagnosis of folliculotropic mycosis fungoides stage IA. Due to an inadequate response to a topical corticosteroid, P-UVA phototherapy was administered, as well as close follow-up, essential for timely treatment of this frequently therapy-resistant disease.","PeriodicalId":30659,"journal":{"name":"Serbian Journal of Dermatology and Venereology","volume":"8 1","pages":"213 - 220"},"PeriodicalIF":0.0000,"publicationDate":"2016-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Folliculotropic Mycosis Fungoides - A Case Report\",\"authors\":\"Ivana Ilijin, Z. Mijuskovic, D. Brasanac, L. Kandolf Sekulovič\",\"doi\":\"10.1515/sjdv-2016-0019\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Abstract Mycosis fungoides (MF) belongs to a group of primary cutaneous T-cell lymphomas, with characteristic small- to medium-sized neoplastic T-lymphocytes with hyperchromatic and cerebriform nuclei. Folliculotropic mycosis fungoides (FMF) represents a variant of mycosis fungoides, which is histologically characterized by folliculotropic T-cell infiltrates, with or without mucinous degeneration of the hair follicles. Clinical features of FMF are characterized by appearance of grouped follicular papules, acneiform lesions, indurated plaques, sometimes tumors, which usually involve the head and neck region. The diagnosis is based on clinical presentation, histopathological and immunohistochemical (IHC) findings of skin biopsy specimens. The treatment of FMF, and generally MF, should be stage-adapted. Case report: We present a case of a 33-year-old male with an eight-month history of erythematous papules on his forehead accompanied by intense pruritus. Histopathological findings showed folliculotropic and perivascular lymphocytic infiltrates. An increased CD4/CD8 ratio of interfollicular lymphocytes with accumulation of Langerhans cell confirmed the diagnosis of FMF. Our patient was diagnosed with an early stage - IA, and P-UVA phototherapy was recommended due to ineffectiveness of prescribed topical corticosteroids that had shortterm effects. Conclusion: Folliculotropic mycosis fungoides represents a diagnostic challenge due to the great diversity of clinical manifestations. We presented a rare case of folliculotropic mycosis fungoides in a young adult, who presented with erythematous papules, accompanied by intense pruritus on the forehead, which lasted for several months. Histopathological and IHC analysis confirmed the diagnosis of folliculotropic mycosis fungoides stage IA. Due to an inadequate response to a topical corticosteroid, P-UVA phototherapy was administered, as well as close follow-up, essential for timely treatment of this frequently therapy-resistant disease.\",\"PeriodicalId\":30659,\"journal\":{\"name\":\"Serbian Journal of Dermatology and Venereology\",\"volume\":\"8 1\",\"pages\":\"213 - 220\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2016-12-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Serbian Journal of Dermatology and Venereology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1515/sjdv-2016-0019\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Serbian Journal of Dermatology and Venereology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1515/sjdv-2016-0019","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Abstract Mycosis fungoides (MF) belongs to a group of primary cutaneous T-cell lymphomas, with characteristic small- to medium-sized neoplastic T-lymphocytes with hyperchromatic and cerebriform nuclei. Folliculotropic mycosis fungoides (FMF) represents a variant of mycosis fungoides, which is histologically characterized by folliculotropic T-cell infiltrates, with or without mucinous degeneration of the hair follicles. Clinical features of FMF are characterized by appearance of grouped follicular papules, acneiform lesions, indurated plaques, sometimes tumors, which usually involve the head and neck region. The diagnosis is based on clinical presentation, histopathological and immunohistochemical (IHC) findings of skin biopsy specimens. The treatment of FMF, and generally MF, should be stage-adapted. Case report: We present a case of a 33-year-old male with an eight-month history of erythematous papules on his forehead accompanied by intense pruritus. Histopathological findings showed folliculotropic and perivascular lymphocytic infiltrates. An increased CD4/CD8 ratio of interfollicular lymphocytes with accumulation of Langerhans cell confirmed the diagnosis of FMF. Our patient was diagnosed with an early stage - IA, and P-UVA phototherapy was recommended due to ineffectiveness of prescribed topical corticosteroids that had shortterm effects. Conclusion: Folliculotropic mycosis fungoides represents a diagnostic challenge due to the great diversity of clinical manifestations. We presented a rare case of folliculotropic mycosis fungoides in a young adult, who presented with erythematous papules, accompanied by intense pruritus on the forehead, which lasted for several months. Histopathological and IHC analysis confirmed the diagnosis of folliculotropic mycosis fungoides stage IA. Due to an inadequate response to a topical corticosteroid, P-UVA phototherapy was administered, as well as close follow-up, essential for timely treatment of this frequently therapy-resistant disease.
期刊介绍:
Serbian Journal of Dermatology and Venereology is a journal of the Serbian Association of Dermatologists and Venereologists. The journal is published in English, quarterly and intended to provide rapid publication of papers in the field of dermatology and venereology. Manuscripts are welcome from all countries in the following categories: editorials, original studies, review articles, professional articles, case reports, and history of medicine.