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Parry-Romberg syndrome (PRS) is a rare form of localized scleroderma mainly affecting children and young adults, characterized by progressive hemifacial atrophy due to shrinkage and degeneration of tissues beneath the skin. Lichen sclerosus et atrophicus (LSA) is a rare chronic inflammatory skin disease that mainly affects preadolescent and perimenopausal females with anogenital and extragenital localization. Herein, we present a case of the coexistence of these two rare entities in a young adolescent female in the lower half of the face. To our knowledge, although there are numerous cases in the literature describing the coexistence of LSA and localized scleroderma, similar cases of the coexistence of PRS and LSA in the same site have not been discussed. [ABSTRACT FROM AUTHOR]

Although anti-TNFα agents have revolutionized the treatment of many inflammatory diseases, various concerns have been reported regarding the risks of cancer development, as well as acceleration of the progression of subclinical, preexisting malignancies. In this case series, we investigated the provocative effect of anti-TNFα drugs in the development of cutaneous mycosis fungoides (MF)-like lymphoproliferative reactions. We describe five patients aged between 25–63 diagnosed with autoimmune disorders (psoriatic arthritis – one patient, Crohn's disease – one patient and ankylosing spondylitis – three patients) who received anti-TNFα agents before the development of a cutaneous lymphoproliferative reaction. Histological and immunophenotypical analysis was typical for mycosis fungoides in all of them. Anti-TNFα agents were stopped with regression of the skin rash. A direct effect of anti-TNFα agents in the development of lymphoproliferative reactions (including MF) is suggested and further analyzed. Treatment cessation can be therapeutic. [ABSTRACT FROM AUTHOR]

The temporal association between anti-COVID 19 vaccination and vitiligo in our patient may suggest a possible contribution of the vaccine to the development of the disease. Given the widespread vaccination of the population with the novel mRNA vaccines against COVID-19, it is of utmost importance to identify any possible adverse effects they may have. The patient recalled that a few days after the first dose of the vaccine he noticed a few hypomelanotic lesions in his forearms which were very subtle and for which he did not seek medical advice. [Extracted from the article]

This article discusses a study conducted in Greece on primary cutaneous lymphoma (PCL) in children and adolescents. The study found that the most common subtypes of PCL observed were lymphomatoid papulosis (LyP) and mycosis fungoides (MF). The Greek cohort showed significant differences compared to the Italian registry, with LyP being relatively uncommon and the folliculotropic variant of MF being overrepresented. The study highlights the need for multicentric studies to capture diverse populations and understand the underlying factors driving these variations. [Extracted from the article]

Primary cutaneous lymphomas (PCL) encompass a heterogeneous group of cutaneous T cell (CTCL) and B cell (CBCL) lymphomas. The scarcity of these clinical entities makes the epidemiological study quite challenging. To date, a few studies of PCL have been conducted in the USA, Europe and Asia. We present epidemiological data of PCL in Greece based on a twelve-year retrospective study. Our study is based on histology reports from the major haemopathology referral centre in Athens, Greece from 2009 to 2020. Clinical data including the type of lymphoma, date of first diagnosis, age at diagnosis and gender were collected and processed. The patients' classification is according to the 2018 update of WHO-EORTC classification for PCL. In total, 1171 new cases with PCL have been registered from the 1st of January 2009 to the 31st of December 2020, consisting of 715 males and 456 females, with a male/female ratio of 1.6. The vast majority was CTCL accounting for 83.2% (n=975) of PCL, followed by CBCL with 196 new cases comprising 16.7% of PCL. The male:female ratio was 1.8 for CTCL and 0.9 for CBCL. Mycosis fungoides (MF) was the most common cutaneous lymphoma accounting for 82.3% of CTCL and 68.6% of all PCL (n=803). Classic MF (n=553, 69%) was the most frequent subtype among MF patients followed by folliculotropic MF (n=138, 17.2%). One hundred and twelve patients with MF were presented with atypical forms of the disease. A male predominance was observed among MF patients with 526 newly diagnosed MF in men and 277 in women and a ratio male/female of 1.9. In contrast to the literature, a decreased number of primary cutaneous CD30+ lymphoproliferative disorders was noticed comprising 8.4% of PCL and 10% of CTCL. The most frequent CBCL was the primary cutaneous follicle centre lymphoma (pcFCBCL). Ninety-five new pcFCBCL cases were recorded accounting for 48.4% of CBCL and 8.1% of PCL. The marginal zone lymphoma (pcMZBCL) was the second most common CBCL with 86 new cases representing 43.8% and 7.3% of CBCL and PCL, respectively. In the present study, an increasing trend for PCL was observed through the years. This increase may reflect the improvements in the detection and classification of these lymphomas in conjunction with an increase of the underlying causative agents. It is noteworthy that in 2020, during the COVID-19 era, there was a downward trend in new PCL diagnoses, mainly those of MF, whilst the diagnoses of CBCL were steady. While the COVID-19 pandemic may be held responsible for this, other reasons may be implicated and it needs further monitoring over the next few years. This report will empower the comparison with epidemiological data of PCL from other countries and the observation of the diagnostic trend contributing to the investigation of pathogenesis of the disease and novel therapies in the near future. [ABSTRACT FROM AUTHOR]

Primary cutaneous lymphomas (PCLs) consist of an heterogenous group of rare malignancies. Cutaneous T-cell lymphomas are the most common among them, with mycosis fungoides being the main representative. The etiology of PCLs is rather unknown; however, there are indications that genetic factors may play a role in CTCL developments [1]. Several studies have examined the incidence of second malignancies in patients with PCLs, as well as their family history of malignancies [2–4]. In our study we examine the association between PCLs and other primary malignancies in patients' personal or family history in Greek population. This retrospective study included 144 patients with histopathologically confirmed diagnosis of PCL and 130 controls matched by gender and age (±4 years). All patients and controls completed a detailed questionnaire regarding sun exposure habits-including personal history of sunburns, use of photo-protective measures, use of sunbeds- exposure to ionizing radiation, smoking and alcohol consumption habits, detailed medical personal history (diseases, medication, other chronic skin conditions, personal history of malignancies, etc.) and family history (chronic skin conditions, malignancies). Statistical analysis of the data was performed using PASW Statistics v25 software. Of the 144 patients included, 78 (54.2%) were males and 66 (45.8%) females (median age 57 years old) and among the 130 control subjects, 70 (53.8%) were males and 60 (46.2%) females (median age 61 years old). Analysis showed that family history of solid organ cancer is related to higher relative risk (OR 1.815) of CL development (p<0.05, 95% CI 1.11–2.95). The two types of solid organ tumors more highlighted were liver cancer (p=0.046) and stomach cancer (p=0.002). Family history of skin cancer as well as hematologic malignancies doesn't seem to affect the risk of CL in statistically significant way (p=0.247 and p=0.093 relatively) in our group. As far as the personal history of other primary malignancies is concerned, statistical analysis showed that there might be a correlation between other malignancies and primary cutaneous lymphomas (p<0.05). At this point we should notice the limitation that the number of patients and controls having a personal history of any type of cancer was small, but it consists a predictor. There are several studies analyzing the correlation of PCLs and second primary cancers. The results of our study showed an association of personal history of cancer with PCLs, as well as a correlation of family history of solid organ malignancy with a higher relative risk of cutaneous lymphoma development. No higher relative risk was detected in subjects with family history of hematologic malignancies. We believe that it is important to be aware of the personal and the family history of patients with PCL, as far as the malignancies are concerned, and try to early recognize possible second malignancies in these patients. [ABSTRACT FROM AUTHOR]

Competing Interests: The authors have no conflict of interest. All the authors have contributed to the intellectual content of this paper.