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Erythrodermic cutaneous T-cell lymphomas (E-CTCLs), including erythrodermic mycosis fungoides (E-MF) and Sezary syndrome (SS), are aggressive and rare variants of CTCL with overlapping clinical and pathological features. Differentiating between E-MF and SS is often challenging due to non-specific symptoms and shared diagnostic criteria. This study aimed to evaluate clinical and laboratory features, progression patterns, prognostic indicators, and survival outcomes in E-CTCL patients, comparing subgroups of E-MF, SS, and secondary SS. A total of 35 patients with E-CTCL were analyzed (6 with E-MF, 15 with SS, 14 with secondary SS). Comprehensive evaluations encompassed clinical and histopathological data, tumor-lymph node-metastasis-blood staging, flow cytometry findings, laboratory parameters, and survival outcomes. Statistical analyses included Kaplan-Meier survival estimates and Cox regression models. Most patients were male (74.3%) and presented with advanced-stage disease (60%). Elevated serum lactate dehydrogenase and beta-2 microglobulin levels were common, particularly in cases of B2 blood involvement. Female sex and eosinophilia were independent predictors of mortality. Lymph node involvement was associated with rapid progression to erythroderma. No significant survival differences were observed among the E-MF, SS, and secondary SS subgroups. E-CTCL remains a diagnostic and therapeutic challenge. Female sex and eosinophilia emerged as key independent prognostic indicators in our study. While survival rates did not significantly differ between the E-MF and SS subgroups, the overall prognosis was poor. Larger prospective studies are needed to refine prognostic models and treatment strategies.

This study evaluated the demographic data, clinical characteristics, treatment approaches, and treatment responses of 43 patients with primary cutaneous CD30 lymphoproliferative disorders. Lymphomatoid papulosis (LyP) was characterized by predominantly papular (94.1%) and generalized (70.6%) lesions, while primary cutaneous anaplastic large-cell lymphoma (pcALCL) presented with tumoral (77.8%) and solitary (77.8%) lesions (p<0.001). Common treatments for LyP included methotrexate (response rate: 78.5%), topical corticosteroids (response rate: 83.3%), and phototherapy (response rate: 85.8%), but relapse rates were high. In pcALCL, complete remission was achieved with all treatments, with no relapses after brentuximab vedotin (BV). Secondary malignancies were noted in 20.6% of LyP cases. Both LyP and pcALCL had a 100% 5-year disease-specific survival rate, although two LyP patients (5.9%) died of secondary malignancies. In conclusion, LyP and pcALCL are both indolent lymphomas, with LyP being more prone to relapse. BV is effective for resistant pcALCL. LyP patients need long-term monitoring due to the risk of secondary malignancies.

Transformed mycosis fungoides (T-MF) is a rare variant of MF with an aggressive course. In this study, we aimed to describe characteristics of MF/Sezary syndrome (SS) patients with transformation. Patients diagnosed with T-MF among MF/SS patients between 2000 and 2014 in a tertiary single center were evaluated retrospectively. Demographic data, clinical data, laboratory data, immunophenotype features, response to treatment, survival, and histopathologic features were analyzed. Among 254 MF patients, 25 patients with T-MF were identified (10.2%) and included in the study. The male-to-female ratio was 2.6/1. The median time between MF diagnosis and transformation was 32 months (range: 0-192). Nine (36%) patients were diagnosed initially with T-MF. Advanced disease stage and high serum lactate dehydrogenase (LDH) levels were indicators of poor prognosis and treatment response. Five of the 18 patients with progressive disease had undergone allogeneic hematopoietic stem cell transplantation (allo-HSCT). Allo-HSCT resulted in complete remission in three (60%) patients. Ten (40%) patients died as a result of disease progression. Mean survival time was 25.2±14.9 (2-56) months after transformation. Advanced stage, high serum LDH levels, and loss of CD26 and CD7 expression in the peripheral blood are poor rognostic factors in T-MF. Treatment-resistant tumors and nodules should be cautionary for T-MF. Patients with T-MF have a shortened survival. Some patients may respond to first-line treatments. However, the majority of patients who do not respond to first-line therapies also are unresponsive to second or third-line therapies. Allo-HSCT may be an alternative option in patients with T-MF.

[LANGUAGE=”English”]Background and Design: Primary cutaneous B-cell lymphomas (PCBCLs) have not been well characterized due to their relatively low incidence and heterogeneous clinical features.Materials and Methods: Data of 29 patients with primary cutaneous marginal zone lymphoma (PCMZL) (n=18), primary cutaneous follicle center B-cell lymphoma (PCFCL) (n=7), and primary cutaneous diffuse large B-cell lymphoma-leg type (PCDLBCL-LT) (n=4) who were followed and treated at Ankara University Faculty of Medicine between 2006 and 2022 were retrospectively evaluated. Clinical characteristics, treatment modalities, and outcomes of all patients were analyzed.Results: Our study supports the data that there are geographical differences in the distribution of B-cell lymphomas and that PCMZL is predominant in Asian countries compared to Europe. The mean age at the time of diagnosis in the PCDLBCL-LT group was significantly older than the indolent PCBCL group (p=0.022). The lesion size was predominantly less than 2 cm in PCMZL, between 2 and 5 cm in PCFCL, and larger than 5 cm in the PCDLBCL-LT group (p=0.006). While skin-directed treatments were the most common approach in the PCMZL and PCFCL groups, multi-agent chemotherapy was the preferred treatment in the PCDLBCL-LT group (p=0.003 and p=0.001, respectively). No significant correlation was found between age, gender, clinical features of the lesion, cutaneous lymphoma international prognostic index score, stage and treatment modalities, recurrence, and overall survival. The World Health Organization and European Organization for Research and Treatment of Cancer classification of cutaneous lymphoma remains the primary guideline for understanding the clinical behavior and prognostic parameters of this heterogeneous patient group.Conclusion: Given the relative rarity of these types of lymphoma, our patient collective provides an additional value to the existing literature.[LANGUAGE=”Turkish”]Amaç: Primer kutanöz B-hücreli lenfomalar (PKBHL), nispeten düşük insidansları ve heterojen klinik özellikleri nedeniyle iyi karakterize edilememiştir.Gereç ve Yöntem: 2006-2022 yılları arasında Ankara Üniversitesi Tıp Fakültesi’nde takip ve tedavi edilen primer kutanöz marjinal zon lenfoma (PKMZL) (n=18), primer kutanöz folikül merkez hücreli lenfoma (PKFMHL) (n=7) ve primer kutanöz diffüz büyük B-hücreli lenfoma-bacak tipi (PKDBBHL-BT) (n=4) olan 29 hastanın verileri retrospektif olarak incelendi. Tüm hastaların klinik özellikleri, tedavi yöntemleri ve sonuçları analiz edildi.Bulgular: Çalışmamız B-hücreli lenfomaların dağılımında coğrafi farklılıklar olduğu ve PKMZL’nin Avrupa’ya kıyasla Asya ülkelerinde baskın olduğu verisini desteklemektedir. PKDBBHL-BT grubunun tanı anındaki ortalama yaşı, indolent PKBHL grubundan anlamlı olarak daha ileri bulunmuştur (p=0,022). Lezyon boyutu PKMZL’de çoğunlukla 2 cm’den küçük, PKFMHL’de 2-5 cm arasında ve PKDBBHL-BT grubunda 5 cm’den büyük saptanmıştır (p=0,006). PKMZL ve PKFMHL gruplarında çoğunlukla deriye yönelik tedaviler kullanılırken, PKDBBHL-BT grubunda çok ajanlı kemoterapi sıklıkla tercih edilmiştir (sırasıyla; p=0,003 ve p=0,001). Yaş, cinsiyet, lezyonun klinik özellikleri, kutanöz lenfoma uluslararası prognostik indeks skoru, evre ve tedavi modaliteleri ile nüks ve genel sağkalım arasında anlamlı bir ilişki saptanmamıştır. Dünya Sağlık Örgütü ve Avrupa Kanser Araştırma ve Tedavi Örgütü kutanöz lenfoma sınıflandırması, bu heterojen hasta grubunda klinik davranışı ve prognostik parametreleri anlamak için halen birincil kılavuz olmaya devam etmektedir.Sonuç: Bu tür lenfomaların görece nadirliği göz önüne alındığında, hasta serimiz mevcut literatüre ek bir değer katmaktadır.

Whether machine-learning algorithms can diagnose all pigmented skin lesions as accurately as human experts is unclear. The aim of this study was to compare the diagnostic accuracy of state-of-the-art machine-learning algorithms with human readers for all clinically relevant types of benign and malignant pigmented skin lesions. For this open, web-based, international, diagnostic study, human readers were asked to diagnose dermatoscopic images selected randomly in 30-image batches from a test set of 1511 images. The diagnoses from human readers were compared with those of 139 algorithms created by 77 machine-learning labs, who participated in the International Skin Imaging Collaboration 2018 challenge and received a training set of 10 015 images in advance. The ground truth of each lesion fell into one of seven predefined disease categories: intraepithelial carcinoma including actinic keratoses and Bowen's disease; basal cell carcinoma; benign keratinocytic lesions including solar lentigo, seborrheic keratosis and lichen planus-like keratosis; dermatofibroma; melanoma; melanocytic nevus; and vascular lesions. The two main outcomes were the differences in the number of correct specific diagnoses per batch between all human readers and the top three algorithms, and between human experts and the top three algorithms. Between Aug 4, 2018, and Sept 30, 2018, 511 human readers from 63 countries had at least one attempt in the reader study. 283 (55·4%) of 511 human readers were board-certified dermatologists, 118 (23·1%) were dermatology residents, and 83 (16·2%) were general practitioners. When comparing all human readers with all machine-learning algorithms, the algorithms achieved a mean of 2·01 (95% CI 1·97 to 2·04; p<0·0001) more correct diagnoses (17·91 [SD 3·42] vs 19·92 [4·27]). 27 human experts with more than 10 years of experience achieved a mean of 18·78 (SD 3·15) correct answers, compared with 25·43 (1·95) correct answers for the top three machine algorithms (mean difference 6·65, 95% CI 6·06–7·25; p<0·0001). The difference between human experts and the top three algorithms was significantly lower for images in the test set that were collected from sources not included in the training set (human underperformance of 11·4%, 95% CI 9·9–12·9 vs 3·6%, 0·8–6·3; p<0·0001). State-of-the-art machine-learning classifiers outperformed human experts in the diagnosis of pigmented skin lesions and should have a more important role in clinical practice. However, a possible limitation of these algorithms is their decreased performance for out-of-distribution images, which should be addressed in future research. None.

[LANGUAGE=”English”]The pathogenesis of mycosis fungoides (MF) is poorly understood, and an immunogenetic mechanism has been suggested to play a role. Human leukocyte antigen (HLA) class II alleles DRB1*11 and DQB1*03 were found to be significantly increased for patients with sporadic and/ or familial MF. The juvenile-onset familial MF is extremely rare. Herein, we report two siblings diagnosed with folliculotropic MF, both exhibiting similar morphology of a unilesional presentation on the flexural region of the arms. Both were positive for HLA-DRB1*04 and HLA-DQB1*03 alleles. The HLA-DQB1*03 allele has been described in familial MF in the literature recently, whereas “HLA-DRB1*04” allele has not been reported previously in familial MF cases.[LANGUAGE=”Turkish”]Mikozis fungoides (MF) patogenezi tam olarak anlaşılamamıştır, immünogenetik mekanizmaların rol oynayabileceği öne sürülmüştür. İnsan lökosit antijeni (HLA) sınıf II allelleri DRB1*11 ve DQB1*03 sporadik ve/veya ailesel MF’si olan hastalarda önemli oranda yüksek bulunmuştur. Juvenil başlangıçlı ailesel MF ise oldukça nadirdir. Burada, her ikisinde de kolların fleksural bölgesinde tek taraflı benzer morfoloji sergileyen ve folikülotropik MF tanısı alan iki kardeşi sunmaktayız. Her iki kardeşde HLA-DRB1*04 ve HLA-DQB1*03 allelleri pozitif bulundu. HLA-DQB1*03 alleli literatürde yakın zamanda ailesel MF’de tanımlanmış olup, “HLA-DRB1*04” alleli daha önce ailesel MF olgularında bildirilmemiştir.

Tumoral melanosis is a rare histopathological phenomenon characterized by confuent dermal nodular aggregates of heavily melanized polygonal cells consistent with melanophages. The initial occurrence of tumoral melanosis always raises the suspicion of a regressed melanocytic proliferation, especially a melanoma. Regression can be recognized by the presence of dermal fibrosis, vascular proliferation, and usually a band-like infiltrate of lymphocytes and melanophages in variable numbers. However, these changes are non-specific, and similar findings can be seen in regression of pigmented epithelial lesions, such as basal cell carcinoma, Bowen’s disease, solar lentigo, and rarely, in mycosis fungoides (1,2). We present a case of tumoral melanosis which arose on a plaque of mycosis fungoides and discuss the possible underlying pathological mechanisms.

Patient's detailed medical history revealed prior use of both conventional and novel anti-tumor necrosis factor-alpha (anti-TNF-a) treatments (methotrexate 15 mg-20 mg/week for four years, etanercept 50 mg/week for two years, adalimumab 40 mg every two weeks for two years, and infliximab 5 mg/kg in usual regimen for two years). Because of refractory psoriatic arthritis; golimumab (50 mg/month subcutaneous) was started after two years of the biologic drug-free period. During the past three decades, use of biologic agents in psoriasis and psoriatic arthritis resulted in better control of disease activity and improved quality of life. Psoriasis patients receiving systemic immunomodulatory drugs must be followed up closely and screening for latent viral infections before initiating the biologic therapy may help in follow-up.

A 49-year-old male was admitted to our department with a 3-year history of pruritus and severe xerosis. Dermatological examination revealed squamation and ichthyosis with dark lamella involving the trunk and extremities (Figure 1). Physical examination revealed bilateral lower extremity edema.