Hypopigmented mycosis fungoides: a review of its clinical features and pathophysiology

Furlan, Felipe Favoretto; Sanches, José Antônio

doi:10.1590/abd1806-4841.20132336

Cited by 67 publications

(107 citation statements)

References 44 publications

(87 reference statements)

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“…Hipopigmente MF'de histopatolojik olarak; fokal parakeratoz, epidermotropizm gösteren vakuollü sitoplazmalı, hiperkromatik, iri nükleuslu, halo ile çevrelenmiş atipik lenfoid hücreler, Pautrier mikroapseleri, üst dermiste spongioz olmaksızın lenfositik infiltrasyon veya orta-şiddette dermal lenfositik infiltrasyon görülmektedir (2,5). Ayrıca hafif psoriaziform hiperplazi, interfaz dermatit gibi dermoepidermal bileşkede vakuoler dejenerasyon, folikülotopizm, melanin inkontinansı ve papiller dermiste melanofajlar görüldüğü de bildirilmiştir (2,5,6).…”

Section: Discussionunclassified

“…Ayrıca hafif psoriaziform hiperplazi, interfaz dermatit gibi dermoepidermal bileşkede vakuoler dejenerasyon, folikülotopizm, melanin inkontinansı ve papiller dermiste melanofajlar görüldüğü de bildirilmiştir (2,5,6). Olgumuzda da histopatolojik olarak özellikle bazal tabakada olmak üzere epidermotropizm gösteren vakuollü sitoplazmalı, hiperkromatik, iri nükleuslu atipik lenfoid hücreler, yer yer Pautrier mikroapseleri, üst dermiste perivasküler hafif lenfositik infiltrasyon görülerek histopatolojik olarak MF tanısı kondu.…”

Section: Discussionunclassified

“…Hipopigmente MF tanısında immünfenotipik hücre marker'lerinin kullanımı tartışmalıdır (2). Klasik MF lezyonlarında epidermotropizm gösteren hücrelerde CD4+ T hücre hakimiyeti bulunurken hipopigmente MF'de ise çoğunlukla CD8+ T hücreleri baskın olarak bulunmaktadır.…”

Section: Discussionunclassified

“…Hipopigmente MF tanısı ancak klinik ve histopatolojik korelasyon ile konulabilir (2,4,8). Bizim olgumuz daha önce deri biyopsisi alınmaması ve tanının geç konması nedeni ile histopatolojinin tanıdaki önemini vurgulayan güzel bir örnektir.…”

Section: Discussionunclassified

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Hypopigmented Mycosis Fungoides: Case Report

Kurmuş¹,

Keseroglu²,

Gönül³

et al. 2018

tdd

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Mikozis fungoides (MF) primer kutanöz T hücreli lenfomaların en sık görülen tipidir. Hipopigmente MF nadir görülen atipik bir klinik varyant olup daha çok Afrika, Hindistan, Latin Amerika ve Asya kökenli koyu tenli bireylerde, özellikle çocuk ve genç erişkinlerde görülmektedir. Beyaz ırkta çok nadir görülmekle birlikte hastalar genellikle gövde ve proksimal ekstremitelere dağılan asemptomatik, düzensiz sınırlı, skuamsız hipopigmente yamalar ile başvurmaktadırlar. Gövde ve üst ekstremitelere dağılan yaygın beyaz lekeler şikayeti ile başvuran, klinik ve histopatolojik olarak hipopigmente MF tanısı konan yirmi beş yaşında kadın olgu nadir görülmesi nedeniyle sunulmuştur. Öz AbstractMycosis fungoides (MF) is the most common type of primary cutaneous T-cell lymphoma. Hypopigmented MF is a rare atypical clinical variant and is mostly seen in dark-skinned individuals of Africa, India, Latin America and Asian origin, especially in children and young adults. As well as it is seen very rare in Caucasians, patients usually consult with asymptomatic, irregular, non-squamous hypopigmented patches which distributed in the trunk and proximal extremities. A 25-year-old female patient clinically and histopathologically hypopigmented MF who presented with diffuse white stains on the trunk and upper extremities was presented because of its rarity.

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Section: Discussionunclassified

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Hypopigmented Mycosis Fungoides: Case Report

Kurmuş¹,

Keseroglu²,

Gönül³

et al. 2018

tdd

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“…In hypopigmented MF lesions, the prevalence of CD8+neoplastic lymphocytes (due to their cytotoxic action) would lead to dysfunction of the melanocytes, altering melanin production and distribution and culminating in the formation of clinically hypochromic lesions 40 . It is an indolent disease, and treatment with topical corticosteroids, topical nitrogen mustard, or phototherapy (either UVBnb or PUVA) can induce clinical remission 41 . …”

Section: Hypopigmented Mfmentioning

confidence: 99%

Cutaneous lymphomas: A review

Miyashiro¹,

Sanches²

2015

Cumhuriyet Med J

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SUMMARYSkin may be affected by non-Hodgkin lymphomas, and it is the second most frequently involved extranodal organ, after the gastrointestinal tract. Cutaneous lymphomas may originate from T, B, or NK lymphocytes. Diagnosis is difficult, and knowledge of these diseases is important to ensure their detection and adequate treatment and follow-up. Clinical picture is heterogeneous, and histology is essential to confirm the diagnosis. The classification is given by the correlation between clinical findings, histology and immunophenotyping. Therapeutic options include skin-directed therapies, immunomodulatory or low-dose immunosuppressive drugs. Rare cases of aggressive disease require systemic multidrug chemotherapy. The purpose of this review is to describe the ontogeny of T, B and NK lymphocytes, and to detail the most accepted classification of cutaneous lymphomas, proposed by the World Health Organisation and European Organisation for Research and Treatment of Cancer. Treatment of cutaneous lymphomas will be briefly discussed.

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Mycosis Fungoides in Children and Adolescents

et al. 2021

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IMPORTANCE Comprehensive data on childhood mycosis fungoides (MF) is scarce. OBJECTIVE To describe clinical features, immunophenotypes, various treatment options, and prognosis of MF in children and adolescents. EVIDENCE REVIEW This systematic review searched MEDLINE via PubMed, Embase, Cochrane, and Scopus databases in October 2019. The search terms included mycosis fungoides, infant, children, and adolescent.No filter for the publication period was used, but studies written in a language other than English were excluded. Reference lists of the relevant articles were also searched manually. Case series and case reports were included if data on childhood MF were extractable. The Asan Medical Center database for cases of childhood MF was also searched. Patients were treated from January 1, 1990, to July 31, 2019, and were younger than 20 years at the time of diagnosis. The methodologic quality of the included studies was assessed with items from the Newcastle-Ottawa scale. Data were analyzed from December 9, 2019, to September 4, 2020.FINDINGS A total of 571 unique patients were included. The mean (SD) age at diagnosis was 12.2 (4.2) years; at onset, 8.6 (4.2) years. The female-to-male ratio was 1:1.6 (350 male patients [61.3%]). Among 522 patients with data available at diagnosis, stage 1 disease constituted 478 cases (91.6%), followed by stage 2 (39 [7.5%]) and stage 4 (5 [1.0%]). Among the 567 patients with data available, the most common variant of MF was the hypopigmented form (309 [54.5%]), followed by classic MF (187 [33.0%]). The MF lesions were predominantly the CD4 + and CD8 + immunophenotype in 99 (49.5%) and 79 (39.5%) of 200 patients, respectively. Among the treatments, narrowband UV-B was the most frequently used (150 of 426 [35.2%]). Most patients were alive with the disease (185 of 279 [66.3%]); 83 of 279 (29.8%) were in complete remission; and 11 of 279 (3.9%) had died by the last follow-up. A longer time from onset to diagnosis (hazard ratio [HR], 1.24; 95% CI, 1.06-1.45), granulomatous slack skin (HR, 12.25; 95% CI,, granulomatous MF (HR, 14.59; 95% CI, 1.31-162.00), a history of organ transplant (HR, 10.15; 95% CI, 0.98-105.37), and stage 2 disease at the time of diagnosis (HR, 10.22; 95% CI, 2.94-35.50) were associated with worse outcomes. CONCLUSIONS AND RELEVANCEThe findings of this review suggest that there is often a significant delay until the establishment of a correct diagnosis of childhood MF, which may be detrimental to the prognosis.

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Hypopigmented mycosis fungoides: a review of its clinical features and pathophysiology

Cited by 67 publications

References 44 publications

Hypopigmented Mycosis Fungoides: Case Report

Hypopigmented Mycosis Fungoides: Case Report

Cutaneous lymphomas: A review

Mycosis Fungoides in Children and Adolescents

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