Folliculotropic Mycosis Fungoides Research Articles

Atypical Presentation of Lichen Planopilaris: Presentation of Two Cases and Review

Lichen Planopilaris (LPP) is an uncommon scalp disorder of unknown etiology and prevalence. It is thought to be an autoimmune process triggered by unknown genetic and/or environmental factors that attack hair follicles of the scalp. LPP has been reported to mimic or present in association with various autoimmune diseases and immunomodulatory therapies. We present two atypical case of LPP in Caucasian patients; the first is a patient with generalized pruritus, skin eruptions, and scalp hair loss. Biopsy of the lesions revealed exocytosis of atypical lymphocytes at the Dermo-Epidermal Junction (DEJ) and formation of small Pautrier’s microabscesses in the interfollicular epidermis as well as a robust lymphocytic inflammatory infiltrate with destruction of perifollicular appendages. This led us to the diagnosis of LPP-like Folliculotropic Mycosis Fungoides (FMF). The second case is a patient with a history of Systemic Lupus Erythematosus (SLE) who presented clinically with perifollicular erythema and alopecic patches, with loss of follicular ostia on the frontal and vertex scalp and evidence of follicular tufting. Histologically, she had decreased number of follicles with a peri-infundibular lymphocytic infiltrate and vacuolization at the Dermoepidermal Junction (DEJ) and there was also thickening of the basement membrane highlighted with a periodic acidic Schiff stain. This led to a diagnosis of SLE/LPP overlap. Keywords: Lichen Planopilaris (LPP); Cicatricial; Alopecia; Frontal Fibrosing Alopecia (FFA), Folliculotropic Mycosis Fungoides (FMF), Lichen Planus (LP).

Mycosis Fungoides in Children and Adolescents: A Series of 23 Cases

BackgroundMycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma in adults and children. The prevalence has increased in some countries, but no descriptive studies of MF in the pediatric population have been done in Colombia to date. MethodsA combined prospective–retrospective study of 128 patients with a diagnosis of MF confirmed by the dermatology department and dermatopathology laboratory of Universidad de Antioquia between 2008 and 2017. We describe the clinical and histopathologic variants, response to treatment, and progression of the disease in 23 patients under 18 years of age. ResultsThe pediatric cases of MF accounted for 18% of all the cases on record. The median age of onset of lesions was 9 years, the median age at diagnosis was 11 years, and the median time between onset of lesions and diagnosis was 2 years. All patients were in early stages of the disease. Hypopigmented MF was the most common clinical presentation (in 52.2%), followed by classical MF (in 30.4%). Folliculotropic MF was identified in 17.4%. All patients were treated with topical corticosteroids and phototherapy. One patient received chemotherapy while still in the early stage of disease. Complete remission was achieved in 59.1% and a partial response in 40.9%. Only 2 patients remained asymptomatic for 5 years. ConclusionWe found hypopigmented MF to be the most common clinical presentation in patients under 18 years of age. The disease did not progress to advanced stages in any of the patients, although recurrence after treatment interruption was common.

Stage-dependent Increase in Expression of miR-155 and Ki-67 and Number of Tumour-associated Inflammatory Cells in Folliculotropic Mycosis Fungoides.

Recent studies suggest that folliculotropic mycosis fungoides (FMF), the most common variant of mycosis fungoides (MF), presents with 2 distinct clinicopathological stages: early indolent stage and more aggressive advanced/tumour stage. To further characterize these stages, miR-155 expression was studied with qRT-PCR and found to be significantly higher in biopsies of tumour-stage FMF compared with early-stage FMF and inflammatory dermatoses. There was no statistically significant difference in miR-155 expression between early-stage FMF and early-stage MF, nor between tumour-stage FMF and tumour-stage MF. Immunohistochemical analysis revealed a significantly increased number of dermal Ki-67+ proliferating lymphocytes in tumour-stage FMF, together with an increased number of CD20+ B cells and CD68+ macrophages compared with early-stage FMF. Thus, similar to classic MF, miR-155, Ki-67 tumour cell immunoreactivity, and certain tumour-infiltrating inflammatory cells are differentially expressed in early-vs tumour-stage FMF. The results of this study corroborate the notion that FMF presents with 2 distinct stages.

Pilot Study of a Novel Therapeutic Approach for Refractory Advanced Stage Folliculotropic Mycosis Fungoides.

Folliculotropic mycosis fungoides is a variant of cutaneous T-cell lymphoma characterized as having a folliculocentric infiltrate of malignant T cells along with a worse prognosis in comparison to the epidermotropic variants. Patients with advanced forms of folliculotropic mycosis fungoides are often poorly responsive to both skin-directed as well as to systemic therapies. We report here a high response rate using a novel therapeutic regimen combining interferon gamma, isotretinoin in low dose and topical carmustine, and in some cases concomitant skin-directed therapies, among 6 consecutive patients with refractory folliculotropic mycosis fungoides with stages IB through IIIB who had previously failed both topical and systemic therapies. The potential mechanisms of this multimodality approach are discussed.

Plaque stage folliculotropic mycosis fungoides: histopathologic features and prognostic factors in a series of 40 patients.

BackgroundFolliculotropic mycosis fungoides (FMF) is a distinct variant of mycosis fungoides. Recent studies recognized indolent and aggressive subgroups of FMF, but there is controversy how patients presenting with plaques should be classified. The present study describes the histopathologic features of 40 FMF plaques. The aim of the study was to identify risk factors for disease progression and poor outcome in this group.MethodsClinical, histopathological, and immunophenotypical data from 40 patients with plaque stage FMF were reviewed and analysed for risk factors for disease progression and survival.ResultsAfter a median follow‐up of 80 months, disease progression occurred in 20 of 40 patients. Percentage of atypical cells, cell size, percentage of Ki‐67+ cells, and co‐existent interfollicular epidermotropism, but not the extent of perifollicular infiltrates, were associated with disease progression and reduced survival, while extensive follicular mucinosis was associated with increased survival.ConclusionsThis study underlines that FMF patients presenting with plaques represent a heterogeneous group and that a subgroup of these patients may have an indolent clinical course. It further shows that histological examination is a valuable tool to differentiate between indolent and aggressive disease.

The different faces of mycosis fungoides: results of a single-center study.

Mycosis fungoides (MF) accounts for the majority of cutaneous lymphomas. Apart from the predominant Alibert-Bazin type, several clinicopathological variants of diverse prevalence and biological behavior have been described. Data on clinical and epidemiological aspects of MF clinical subtypes are still weak. To outline the clinical and epidemiological profile of the different MF types in a large volume of Greek patients. Retrospective analysis of 688 MF cases treated in our lymphoma clinic. Epidemiological, clinical, pathological, and immunohistochemical data were retrieved. Six-hundred and thirty-six patients (416 males, 220 females) were included. The mean age at diagnosis was 60.2years; the mean duration of disease prior to diagnosis was 63.2months. Early-stage MF (I-IIA) involved 475 cases (74.7%). The prevalent type was classical MF (68.5%), followed by folliculotropic (17%), poikilodermic (5.5%), and psoriasiform (4.7%) MF. Atypical MF lesions as the sole manifestation of folliculotropic mycosis fungoides (FMF) - alopecia areata-like lesions (n=10), keratosis pilaris-like lesions (n=9) or acneiform rash (n=4) - were also observed. Both poikilodermic and folliculotropic subtypes mainly involved younger patients. A significant diagnostic latency concerning poikilodermic and psoriasiform MF cases was recorded. Only 23 (3.3%) cases were of juvenile onset, with classical and poikilodermic MF equally affecting this age group, closely followed by FMF. Our study presents the whole clinical-epidemiological spectrum of MF in a large Greek cohort. The high prevalence of atypical MF manifestations characterized by early onset and indolent clinical course stood out among our FMF sample.

Syringotropic Mycosis Fungoides: A Variant of Folliculotropic Mycosis Fungoides or a Distinct Entity?

Syringotropic mycosis fungoides (MF) is classified under folliculotropic MF. Although there is significant overlap between the 2, this study demonstrates that folliculotropism is frequently present in syringotropic MF, and when not present, the specimen did not include a follicle to examine. In addition, few of the pathology reports mentioned folliculotropism or syringotropism, although this is an important prognostic feature.

Two distinct variants of mycosis fungoides (MF): Folliculotropic MF and erythrodermic MF.

Mycosis fungoides (MF) is the most frequent type of cutaneous T-cell lymphoma. Folliculotropic MF (fMF) and erythrodermic MF (eMF) are two distinct variants of MF. Both variants have been considered aggressive and most cases are less responsive to standard skin-directed therapies than classical MF. We, however, experienced many cases with fMF or eMF who showed indolent clinical courses. In this article, we reviewed 10 cases with fMF and 13 cases with eMF who came to our department between 2005 and 2017. In patients with fMF, monotherapy with topical corticosteroid was effective in two cases (20%) and ultraviolet phototherapy with oral retinoid controlled disease activity in two cases (20%). Five patients with eMF (38%) responded well to ultraviolet phototherapy. In conclusion, patients with early fMF and a subgroup of eMF patients have an indolent disease course, as was proposed among the specialists. Skin-directed therapies are preferable rather than aggressive treatment in those cases.

Exploratory Assessment of Oxygen Flow–Assisted Cutaneous Administration of Methotrexate for Superficial Basal Cell Carcinoma, Mycosis Fungoides, and Extramammary Paget Disease

The molecular weight of methotrexate (MTX) makes cutaneous penetration difficult. Oxygen flow could enhance the skin permeation of MTX diluted in the proprietary LP3 carrier system. This pilot study aims to assess the efficacy, safety, and tolerance of oxygen flow-assisted LP3-MTX3% for treating superficial skin cancers. Patients with superficial basal cell carcinoma (n= 12), extramammary Paget disease (n= 5), classic mycosis fungoides (MF; n= 10), and folliculotropic MF (n= 6) were included in the study and were treated with four weekly applications of oxygen flow-assisted LP3-MTX3%. Photographs and biopsies were performed before and one month after treatment. At one month after treatment, the mean superficial basal cell carcinoma erythema-crusting-thickness clinical score, the extramammary Paget disease erythema-oozing-scaling/hyperkeratosis-pain/pruritus clinical score, and the modified composite assessment of index lesion severity classic MF and folliculotropic MF scores were improved by 77.5% ± 17.1% (P < 0.0001), 66.7% ± 22.9% (P= 0.011), 51.3% ± 32.2% (P= 0.0007), and 27.8% ± 32.0% (P= 0.086), respectively. At one month after treatment, histology revealed partial and total clearances for superficial basal cell carcinoma (1/12, 11/12), extramammary Paget disease (4/5, 1/5), classic MF (8/10, 2/10), and folliculotropic MF (6/6, 0/6). Tolerance was excellent and no pain was observed. MTX was never detectable in serum at baseline and 1, 2, 3, 8, 24, 48, and 72 hours post-treatment. In conclusion, the interesting therapeutic efficacy of oxygen flow-assisted LP3-MTX3% for treating superficial basal cell carcinoma, extramammary Paget disease, and MF lesions prompts further studies on a larger scale.

亲毛囊性并肉芽肿性蕈样肉芽肿一例

亲毛囊性并肉芽肿性蕈样肉芽肿一例

A retrospective analysis of clinicopathologic features and treatment response rates in patients with folliculotropic mycosis fungoides in a series of 29 cases

A retrospective analysis of clinicopathologic features and treatment response rates in patients with folliculotropic mycosis fungoides in a series of 29 cases

A case of folliculotropic mycosis fungoides

A 24-year old man complained that he had been suffered from hair lost on his right occipital scalp for nineteen years. Physical examination showed a localized irregular erythema with hair lost on his right occipital scalp. The lesion was covered with tiny scale,and its temperature was slightly high. Few residual vellus hairs could be seen around the erythema. Histopathological examination showed inflammatory cells (mainly lymphocyte ) infiltrated into follicles and epidermis. Pautrier microabscess could be seen within the follicles. The infiltrated cell were positive for CD3,CD45Ro,LCA,however, negative for CD20 and alcian blue stain. Diagnosis of folliculotropic mycosis fungoides was made.

Revisiting Imiquimod for Treatment of Folliculotropic Mycosis Fungoides: A Case Report and Review of the Literature.

Folliculotropic mycosis fungoides (FMF) is an aggressive variant of mycosis fungoides (MF) characterized by infiltration of the hair follicle epithelium by neoplastic T cells. FMF demonstrates poor response rates to standard skin-directed therapies such as phototherapy and topical corticosteroids. Imiquimod, an immunomodulatory agent that stimulates the antitumor immune response, has been used successfully in treatment of early-stage MF. We report a 21-year-old patient with unilesional FMF who achieved clinical remission with imiquimod application. This case highlights a potential for use of imiquimod as a treatment option for patients with FMF and limited skin involvement.

Micosis fungoide en niños y adolescentes: descripción de una serie de 23 casos

BackgroundMycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma in adults and children. The prevalence has increased in some countries, but no descriptive studies of MF in the pediatric population have been done in Colombia to date. MethodsA combined prospective-retrospective study of 128 patients with a diagnosis of MF confirmed by the dermatology department and dermatopathology laboratory of Universidad de Antioquia between 2008 and 2017. We describe the clinical and histopathologic variants, response to treatment, and progression of the disease in 23 patients under 18 years of age. ResultsThe pediatric cases of MF accounted for 18% of all the cases on record. The median age of onset of lesions was 9 years, the median age at diagnosis was 11 years, and the median time between onset of lesions and diagnosis was 2 years. All patients were in early stages of the disease. Hypopigmented MF was the most common clinical presentation (in 52.2%), followed by classical MF (in 30.4%). Folliculotropic MF was identified in 17.4%. All patients were treated with topical corticosteroids and phototherapy. One patient received chemotherapy while still in the early stage of disease. Complete remission was achieved in 59.1% and a partial response in 40.9%. Only 2 patients remained asymptomatic for 5 years. ConclusionWe found hypopigmented MF to be the most common clinical presentation in patients under 18 years of age. The disease did not progress to advanced stages in any of the patients, although recurrence after treatment interruption was common.

Cutaneous lymphomas-An update 2019.

Primary cutaneous lymphomas (CL) are the second most common form of extranodal lymphomas. Cutaneous T-cell lymphomas represent the majority. They are classified according to the WHO classification 2017 and the updated WHO-EORTC 2018 published in the fourth edition of the WHO classification for Skin Tumors monograph. Primary cutaneous acral CD8+ T-cell lymphoma and EBV-positive mucocutaneous ulcer have been listed as new provisional entities. Moreover, the histological and genetic spectrum of lymphomatoid papulosis has been expanded. Recently, prognostic subtypes were delineated for some entities and subtypes of CL such as folliculotropic mycosis fungoides and marginal zone lymphoma. Since CL show overlapping histological features, clinico-pathological correlation is of outmost importance for the diagnosis. Recent studies revealed new biomarkers and genetic alterations underlying the pathogenesis of CL. Moreover, targeted therapies have widened the treatment options particularly for aggressive lymphomas.

PUVA 療法で寛解した folliculotropic mycosis fungoides の 1 例

PUVA 療法で寛解した folliculotropic mycosis fungoides の 1 例

Influence of the phenotype on mycosis fungoides prognosis, a retrospective cohort study of 160 patients

Mycosis fungoides (MF) typically has a CD4+ CD8- T-cell phenotype. Rare cases of CD4- CD8+ , CD4- CD8- , or CD4+ CD8+ immunophenotypes have been described. Little is known about the impact of MF immunophenotypes on disease behavior. We conducted a retrospective cohort study to review all cases of MF from 2007 to 2017 from Sunnybrook Health Sciences Centre, Toronto, Canada. CD4+ CD8- (Group 1) was compared to the three less common subtypes (Group 2) with respect to stage at diagnosis, progression, and transformation. Potential confounding factors (demographic, clinical, and laboratory parameters) were assessed. A total of 160 patients with confirmed MF were analyzed, including 126 CD4+ CD8- MF (79%), 26 CD4- CD8+ MF (16%), six CD4+ CD8+ MF (4%), and two CD4- CD8- MF (1%). Both groups were similar with respect to demographics and laboratory parameters at the time of diagnosis. There was no difference between patients with late stage disease (10% vs. 9%) for groups 1 and 2, respectively (P=0.901). There was no statistically significant difference either in 5-year progression (27.7% vs. 23.5%, P=0.283) or transformation (16.2% vs. 17.3%, P=0.350) estimates. We did find that atypical immunophenotypes presented with different clinical morphologies and were less likely to require systemic therapy. Our large cohort study indicates that atypical MF immunophenotypes do not seem to influence prognosis. Hypopigmented MF was more frequent in the CD4- CD8+ group while folliculotropic MF was exclusively seen in the CD4+ CD8- group. We believe that cases of CD8+ MF with aggressive behavior described in the literature represent misclassified primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma. The small number of patients included in the study is a limiting factor.

052 - Prognostic value of histopathologic features in folliculotropic mycosis fungoides presenting with plaques

052 - Prognostic value of histopathologic features in folliculotropic mycosis fungoides presenting with plaques

026 - Demodex-induced follicular mucinosis of the head and neck mimicking folliculotropic mycosis fungoides

026 - Demodex-induced follicular mucinosis of the head and neck mimicking folliculotropic mycosis fungoides

A case of folliculotropic mycosis fungoides with leonine facies

A case of folliculotropic mycosis fungoides with leonine facies