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Background: Efficacy and safety of mogamulizumab, a monoclonal antibody directed against C‐C chemokine receptor 4, were demonstrated in a previous multinational clinical trial conducted in patients with previously treated cutaneous T‐cell lymphoma (CTCL): Sézary syndrome (SS) or Mycosis Fungoides (MF). Objectives: The real‐world French OMEGA study aimed to describe effectiveness and tolerability of mogamulizumab in adult patients with CTCL, overall and according to the disease (SS or MF). Methods: In this retrospective study, patients treated with mogamulizumab for SS or MF were included from 14 French expert centres. The overall response rate (ORR) under treatment was described (primary criterion), as well as treatment use and safety data. Results: The 122 analysed patients (69 SS, 53 MF) were aged 66.6 ± 12.1 years at mogamulizumab initiation, and their median disease duration was 2.5 years (IQR: 1.3–5.6). Prior to treatment start, they received a median of three systemic CTCL therapies (2–5). Overall, 77.8% of patients suffered from advanced disease (Stage IIB–IVB), with frequent blood (B1/B2) involvement (67.5%). Over the treatment period (median: 4.6 months, 2.1–7.2), 96.7% of patients received all the planned mogamulizumab infusions. Among the 109 patients evaluable for effectiveness, ORR was 58.7% (95% CI [48.9–68.1]) overall, 69.5% [56.1–80.8] in SS and 46.0% [31.8–60.7] in MF. Compartmental response in the blood was observed in 81.8% [69.1–90.9] of SS patients. Skin responses were observed in 57.0% [47.0–66.5] of patients overall, 66.7% [52.9–78.6] in SS and 46.0% [31.8–60.7] in MF. The most common serious adverse drug reactions were rash (8.1% of patients) and infusion‐related reactions (2.4%) which led to treatment discontinuation in 7.3% and 0.8% of patients, respectively. One patient with SS died from mogamulizumab‐related tumour lysis syndrome. Conclusions: This large French study confirmed the effectiveness and tolerability of mogamulizumab in SS and MF patients in routine medical practice. [ABSTRACT FROM AUTHOR]

Summary: Background: Lymphomatoid papulosis (LyP) type D (LyP D) and type E (LyP E) have recently been described in small series of cases or isolated case reports. Aim: To further describe the clinical and histological features of LyP D and E based on a retrospective multicentre study. Methods: The clinical and histopathological features of 29 patients with an initial diagnosis of LyP D or LyP E were retrospectively assessed using standardized forms. Results: After exclusion of 5 cases, 24 patients (14 LyP D, 10 LyP E) were enrolled in the study. The median follow‐up was 2.5 years (range 1 month to 13 years). LyP D was characterized by multiple recurrent self‐regressing small papules that developed central erosion or necrosis, whereas LyP E presented as papulonodular lesions that rapidly evolved into necrotic eschar‐like lesions > 10 mm in size. Epidermal changes were more frequent in LyP D, whereas dermal infiltrates were deeper in LyP E. Anaplastic cytology was rare and the DUSP22 rearrangement was never observed. Two patients (8%) had an associated cutaneous lymphoma. Conclusion: LyP D and E have distinct clinical findings and may be associated with other cutaneous lymphomas. [ABSTRACT FROM AUTHOR]

A commonly used assessment is the Psoriasis Area and Severity Index (PASI), with an absolute value of 10 or more indicating severe disease.1 How patients perceive the severity of psoriasis and physicians' evaluations may be discordant, especially when lesions involve visible areas or are associated with itching. [Extracted from the article]

At diagnosis of ECTL, only one patient displayed an enlarged node > 1-5 cm (not biopsied), and the median number of Sézary cells was 1440 per mm SP 3 sp (range 396-4930). Dear Editor, The prognosis of erythrodermic cutaneous T-cell lymphomas (ECTL) depends on lymph node (N) and visceral (M) involvement, and blood stage (B). Pathological data at progression were reviewed for eight patients: skin biopsy (four patients), node biopsy (five patients) and cerebrospinal fluid (one patient). [Extracted from the article]

Abstract: Background: Prurigo is a common primary pruritic condition. Treatment is challenging. Methotrexate (MTX) is effective for the treatment of pruriginous dermatoses, but its use in prurigo has been little studied. Objectives: To investigate the efficacy and safety of MTX in the treatment of difficult‐to‐treat prurigo. Methods: Patients from six university dermatology departments treated with MTX between 2006 and 2016 for difficult‐to‐treat prurigo (i.e. with failure to conventional therapies) were included in this retrospective multicentre study. Patients with other pruritic dermatoses were excluded. Clinical efficacy was recorded after 3, 6 and 12 months of treatment: (i) subjective efficacy, that is, evaluation of the pruritus by the patient and (ii) objective efficacy, that is, assessment of cutaneous lesions by the physician: complete or almost complete remission (CR) (healing of lesions), partial remission (PR) (incomplete improvement of lesions) or failure (no improvement or worsening). The overall response rate (ORR) included CR and PR. Results: Thirty‐nine patients with previous failure of topical steroids, H1‐antihistamine drugs or phototherapy were included. The median weekly dose of MTX was 15 mg (range 5–25 mg). The median follow‐up was 16 months (2–108). The mean time between onset of MTX and objective efficacy was 2.4 ± 1.2 months and the mean duration of response was 19 ± 15 months. The ORR was 91% at 3 months [n = 36, CI 95% (81.2–100.8%), CR 44%], 94% at 6 months [n = 32, CI 95% (85.7–102.2%), CR 56%] and 89% at 12 months [n = 28, CI 95% (77.4–100.6%), CR 57%]. Seven patients stopped MTX because of failure, and five because of the discovery of hepatocarcinoma (n = 1), elevated transaminases (n = 1), infectious pneumonitis (n = 1) or gastrointestinal symptoms (n = 2). Conclusion: Methotrexate is a therapeutic option in difficult‐to‐treat prurigo. [ABSTRACT FROM AUTHOR]

Clinical presentation, therapeutic approach and outcome of primary cutaneous marginal zone B-cell lymphoma presenting as AL amyloidoma of the skin One patient (patient 3) with systemic lupus erythematosus and Sjögren syndrome developed PCMZBCL after abatacept treatment. Two patients received local treatment with very modest improvement: radiotherapy (24 Gy) (patient 3), topical clobetasol, cryotherapy and V-beam laser (patient 1). [Extracted from the article]

Résumé Le vismodegib, inhibiteur de la voie de signalisation hedgehog, est une nouvelle option thérapeutique dans les carcinomes basocellulaires localement avancés (CBCLa) pour lesquels la chirurgie ou la radiothérapie ne sont pas appropriés. Sa prescription doit faire l’objet d’une décision en réunion de concertation pluridisciplinaire. Si les taux de réponse sont élevés avec une efficacité rapide et prolongée, les réponses complètes sont rares. D’autre part, les effets secondaires fréquents peuvent limiter la poursuite du traitement ou faire discuter des traitements séquentiels. Nous rapportons deux observations qui illustrent la gravité potentielle des CBCLa, tumeurs négligées par les patients et leur entourage rendant alors leur prise en charge chirurgicale impossible ou délabrante sans garantie carcinologique. Ces observations illustrent l’indication du vismodegib et la possibilité d’utiliser une radiothérapie en association ou en relais, après obtention d’une réduction tumorale sous vismodegib. Un tel traitement doit être connu des chirurgiens prenant en charge les CBCLa, à la fois comme alternative mais aussi comme possible traitement néoadjuvant dont la place à ce titre est en cours d’évaluation. Summary The vismodegib, inhibitor of the hedgehog signaling pathway, is a new therapeutic option in locally advanced BCC when surgery or radiotherapy are inappropriate. If the response rate is high with rapid and sustained efficacy, complete responses are rare. Furthermore, the common side effects may limit continuous and prolonged treatment and lead to discuss sequential treatments. We report two cases that illustrate the severity of LaCBC, tumors neglected by patients and their families limiting therapeutic choice especially surgery that become impossible and for which vismodegib is indicated. These observations illustrate the possible interest of radiotherapy in combination or after tumor debulking by vismodegib. Vismodegib must be known by surgeons for LaCBC, mainly as an alternative beyond surgery but also as a possible neoadjuvant treatment to surgery that have to be evaluated. [ABSTRACT FROM AUTHOR]

Background Blastic plasmacytoid dendritic cell neoplasm (BPDCN) represents the malignant counterpart derived from plasmacytoid dendritic cells. This rare entity is usually revealed and diagnosed on cutaneous lesions associated or not with a leukaemic component. The prognosis associated with BPDCN is very poor. Objectives To perform a retrospective review of BPDCN cases registered in the French Study Group on Cutaneous Lymphoma database from June 1995 to May 2008. Methods Forty-seven patients were included. Demographic data, initial staging, therapeutic management and outcome were recorded. Results The mean survival was 16·7 months (95% confidence interval 12·6–20·8). Only eight (17%) and one (2%) patients reached respectively 2 and 5 years of survival. Initial spreading of the disease did not represent, in this cohort, a reliable prognosis factor. The outcome was overall influenced by treatment provided. While radiation therapy, monochemotherapy or even polychemotherapy regimens did not significantly affect the course of the disease, the survival of bone marrow transplanted patients was significantly higher. Conclusions Despite the fact that BPDCN is often initially limited to the skin, only an aggressive initial therapy may improve the patients’ prognosis. Local treatments, such radiation therapy, are definitively useless. Regardless of the initial extension of the disease, in our experience only bone marrow transplantation significantly improved the outcome. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented about aggressive intermediate cell histiocytosis successfully treated by 2-chlorodeoxyadesine.

We report a patient with a sodium valproate-induced cutaneous pseudolymphoma, presenting with an erythematous papule, histologically mimicking a non-epidermotropic T-cell lymphoma. Polymerase chain reaction study of the skin biopsy revealed monoclonal rearrangement of the T-cell receptor γ gene. Withdrawal of sodium valproate was followed by regression of the lesion, but 5 months after substitution by carbamazepine, two further papules appeared, with similar histological features and a T-cell clone identical to the initial one. Carbamazepine was stopped and the lesions disappeared without relapse over a 4-year follow-up. Sodium valproate is very rarely responsible for a hypersensitivity syndrome, and our case is the first report of sodium valproate-induced cutaneous pseudolymphoma. The recurrence with carbamazepine may be due to a common effect on T-cell lymphocyte function. The return of the same monoclonal population shows that the recurrence of monoclonal T cells may be observed in benign conditions and is not an exclusive hallmark of cutaneous lymphoma. [ABSTRACT FROM AUTHOR]

A 58-year-old man had long-standing lesions of presumed large plaque parapsoriasis. Following treatment for nodal Hodgkin’s disease (HD), these became more infiltrated, with a diagnosis of mycosis fungoides (MF). A few months later, nodules appeared on the right leg, which was lymphoedematous after inguinal irradiation for HD. Histopathological examination showed CD3+, CD30–, CD15– large pleomorphic lymphocytes, leading to the diagnosis of transformed MF. The cutaneous lesions were successfully treated with topical nitrogen mustard and interferon alfa-2b then methotrexate, but his general health worsened with depression and malaise, without specific neurological symptoms or extracutaneous spreading of the lymphoma. Cerebral computed tomographic scan revealed a cerebellar subdural collection, arachnoid cyst and quadriventricular hydrocephaly, initially considered to be non-specific. After a few weeks, clinical symptoms of intracranial hypertension appeared, and a cerebrospinal fluid (CSF) examination revealed meningeal involvement by the lymphoma. These cells were CD3-negative and the diagnosis was confirmed by polymerase chain reaction (PCR) study, which revealed an identical clonal rearrangement of the T-cell receptor γ gene between cutaneous biopsies and the CSF. Repeated intrathecal injections of methotrexate and cranial irradiation were performed and the patient was still alive after 13 months. This case illustrates the possible meningeal involvement of MF that may be preceded by atypical and mild neurological or psychiatric symptoms, which may be dissociated from the evolution of the cutaneous lesions. Moreover, PCR study may be useful for both diagnosis and monitoring. [ABSTRACT FROM AUTHOR]

Reports on a retrospective study of patients with pityriasis rubra pilaris (PRP) in France from 1973 to 1997. Determination of patients' response to several treatments in order to define the best management of PRP; Suggestion that retinoids should be considered a first-line treatment.

A letter to the editor is presented in response to the article concerning erythema annulare centrifugum revealing chronic lymphocytic leukemia, published within the issue.

Reports on a case of mycosis fungoides (MF) mimicking granuloma annulare. Description of the lesions; Results of skin biopsies; Clinical presentation of granulomatous MF; Descriptio of granuloma annulare.

Introduction Dans le spectre de maladies auto-inflammatoires systémiques, différents syndromes associant un pyoderma gangrenosum (PG), une hidrosadénite suppurée (HS) et des arthrites ont été décrits. Parmi eux, le PASH (PG, acné, HS), le PAPA (arthrite purulente, acné et PG), le PAPASH (arthrite purulente, PG, acné, et HS), le PASS (PG, acné, HS et spondylarthrite ankylosante HLAB27 +) et plus récemment le PsAPASH (arthrites psoriasiques, PG, acné, et HS). Certaines de ces affections sont liées à des mutations du gène codant la protéine proline-sérine-thréonine phosphatase-1 (PSTPIP1) qui est impliquée dans la régulation de la réponse immunitaire innée, pour d’autres, aucune mutation génétique n’a pu être mise en évidence jusqu’à maintenant. Observation Un homme de 50 ans, tabagique, présentait depuis l’âge de 20 ans une HS axillaire et périnéale stade 3 de Hurley associée à une acné modérée du visage. Depuis quelques mois, étaient apparues, d’une part, des lésions pustuleuses des jambes d’évolution ulcéro-nécrotique dont la clinique et l’histologie étaient en faveur d’une dermatose neutrophilique à type de PG et, d’autre part, des arthralgies inflammatoires périphériques (poignets, IPP, genoux, pieds). Les radios ne montraient pas de lésions destructrices, il n’y avait pas de facteur rhumatoïde mais le marqueur HLA B27+ était présent, ceci conduisant au diagnostic de spondylarthrite ankylosante (SPA) périphérique. L’ensemble de la symptomatologie évoquait un syndrome auto-inflammatoire associant une HS, une acné, un PG et une SPA périphérique. L’analyse génétique n’a pas montré de mutation du gène PSTPIP1. Le patient est actuellement traité par adalimumab (40 mg tous les 15 ours) et méthotrexate (20 mg/semaine) avec de bons résultats sur le PG et la SPA, sans efficacité pour l’HS. Discussion Le syndrome auto-inflammatoire que présente notre patient est différent de ceux déjà décrits. En effet, dans le PAPA syndrome, il n’y a pas d’HS associée. Dans le PAPASH et le PsAPASH, il existe un psoriasis cutané ou un rhumatisme psoriasique que n’a pas notre patient. Dans le PASH syndrome, aucune atteinte articulaire n’est rapportée. Notre observation est proche du PASS syndrome associant un PG, une acné, une HS et une SPA HLA B27 + axiale, mais avec une SPA à prédominance périphérique. Sur le plan thérapeutique, l’efficacité des anti-TNF (infliximab, adalimumab) a été rapportée dans quelques cas. Les anti-IL-1 ont également entraîné une amélioration spectaculaire chez plusieurs patients faisant discuter le rôle de cette cytokine dans la physiopathologie de ces syndromes, mais avec récidive à l’arrêt du traitement. Conclusion La description de ces nouveaux syndromes auto-inflammatoires associant le plus souvent des manifestations cutanées et articulaires, parfois digestives, doit permettre de mieux comprendre leur physiopathologie afin d’améliorer leur prise en charge thérapeutique souvent difficile. [ABSTRACT FROM AUTHOR]

Dear Editor, Primary cutaneous anaplastic lymphomas (pcALCLs) commonly present as solitary or grouped skin lesions and have a 5-year specific survival greater than 91%.1-3 One study showed that, in cases of extracutaneous extension, 5-year overall survival (OS) is 41%.4 At diagnosis some patients have skin and locoregional node involvement, but their prognosis is as favourable as that of patients with lesions limited to the skin.1 Optimal treatment for these patients (brentuximab vedotin, multiagent chemotherapies or less intensive therapies such as methotrexate or radiation) is currently unknown.5-7 The aim of our study was to assess the prognosis of patients with pcALCL and regional lymph node involvement at diagnosis according to different treatment regimens. As first-line treatment, eight patients (group 1) received multiagent chemotherapy (anthracycline-based in seven patients and ifosfamide and cyclophosphamide in one patient). We observed no difference in the risk of progression during the follow-up period between patients undergoing moderate-intensity treatment and patients treated with multiagent therapies. [Extracted from the article]

A letter to the editor on necrosis after vertebroplasty is presented.

Presents a letter to the editor about bone marrow biopsy in patients with mycosis fungoides.

Trichoblastic carcinoma is a rare malignant cutaneous adnexal tumor with a risk of local invasion and distant metastasis. As of today, there is no consensus for the treatment of locally advanced or metastatic trichoblastic carcinoma. "AcSé Nivolumab" is a multi-center Phase II basket clinical trial (NCT03012581) evaluating the safety and efficacy of nivolumab in several cohorts of rare, advanced cancers. Here we report the results of nivolumab in patients with trichoblastic carcinoma. Of the eleven patients enrolled in the study, five patients had been previously treated by sonic hedgehog inhibitors. The primary endpoint 12-week objective response rate was 9.1% (N = 1/11) with 1 partial response. Six patients who progressed under previous lines of treatment showed stable disease at 12 weeks, reflecting a good control of the disease with nivolumab. Furthermore, 54.5% of the patients (N = 6/11) had their disease under control at 6 months. The 1-year overall survival was 80%, and the median progression-free survival was 8.4 months (95%CI, 5.7 to NA). With 2 responders (2 complete responses), the best response rate to nivolumab at any time was 18.2% (95%CI, 2.3–51.8%). No new safety signals were identified, and adverse events observed herein were previously described and well known with nivolumab monotherapy. These results are promising, suggesting that nivolumab might be an option for patients with advanced trichoblastic carcinomas. Further studies on larger cohorts are necessary to confirm these results and define the role of nivolumab in the treatment of trichoblastic carcinomas. [ABSTRACT FROM AUTHOR]

In high-risk LA cSCC, approximately 20% of patients (pts) experience local disease recurrence within 5 years after surgical resection and adjuvant radiotherapy (RT). Therefore, improved treatment options are needed. The PD-1 inhibitors pembrolizumab (pembro) and cemiplimab have demonstrated durable antitumor activity in advanced metastatic cSCC. The randomized, double-blind, placebo-controlled, phase 3 KEYNOTE-630 (NCT03833167) trial will evaluate adjuvant pembro in pts with resectable, high-risk, LA cSCC. Eligible pts have histologically confirmed LA cSCC as the primary site of malignancy and have undergone complete macroscopic resection of all disease with ≥1 high-risk feature: histologically involved nodal disease with extracapsular extension, with ≥1 lymph node >2 cm in diameter or ≥2 lymph nodes involved; any gross cortical bone, skull base, and/or skull base foramen invasion; any index tumor with ≥2 of the following: tumor ≥4 cm with >6-mm depth or invasion beyond subcutaneous fat, multifocal perineural invasion for nerves <0.1 mm in diameter (≥3 foci) or any involved nerve ≥0.1 mm in diameter, poor differentiation and/or sarcomatoid and/or spindle cell histology, recurrent disease (recurrence within 3 years in the previously treated area), or satellite lesions and/or in-transit metastases, lymphatic or vascular involvement. Pts must have received adequate postoperative dose of hypofractionated or conventional RT, including a BED EQD2 >48 Gy, have ECOG PS of 0 or 1, and completed adjuvant RT ≥4 and ≤16 weeks from randomization. Pts are required to provide tumor tissue for PD-L1 testing. Eligible pts will be randomly assigned 1:1 to receive pembro 400 mg IV Q6W or placebo for approximately 1 year (≤9 cycles) and will be followed up for as long as 5 years. Stratification factors are extracapsular extension, cortical bone invasion, and prior systemic therapy (all, yes vs no). Placebo pts may switch to pembro treatment (≤18 cycles) if they have a recurrence within 5 years. In the pembo arm, pts may also receive up to 18 cycles of pembro retreatment. Primary end point is RFS per investigator assessment with biopsy confirmation and secondary end points include OS, HRQoL, and safety. Computed tomography/magnetic resonance imaging will be performed at screening and every 12 weeks until end of year 2, then every 6 months until end of 5 years. Adverse events will be monitored throughout the study and for 30 days after treatment end and graded per NCI CTCAE v4.0. Approximately 570 pts will be enrolled at sites in Asia, Australia, Europe, and North and South America. Results of KEYNOTE-630 will elucidate the role of adjuvant pembro among pts with high-risk, LA cSCC. [ABSTRACT FROM AUTHOR]

Background: To investigate the evolution of bone metastases in patients receiving immune checkpoint inhibitors (ICI). Methods: A single-center retrospective study included cancer patients with bone metastases treated with ICI at our institution between January 2014 and September 2019. Clinical and biological data were collected from medical records and independent expert review of imaging was performed. Target and non-target lesions were identified and followed up to 1 year. Patients were then classified as bone responder or non-responder. Comparisons between groups were performed with Student's t test or Mann–Whitney test. Results: Among 1108 patients screened, 192 patients had bone metastases and 48 patients were included in the final analysis, with lung cancer, renal carcinoma and melanoma as most represented cancer type. Half of the patients experienced stability, condensation or peripheral sclerosis of bone lesions. Initial progression before stabilization with or without sclerosis of bone lesion occurred for 19% of patients (pseudoprogression). There was an association between bone response and global oncological outcomes. Bone responder patients had a significant decrease in morphine and co-analgesic prescription as well as a significant decrease in alkaline phosphatases compared to non-responder patients. Conclusion: Bone response was observed in half of patients with available imaging and follow-up after 3 months of ICI treatment, with sclerosis observed in one-third of bone lesions at month 3, in all tumor types. Up to 20% of patients experienced a pseudoprogression of bone lesions such as previously described in primary tumor and other metastatic sites. Bone response was associated with improvement of pain and survival. [ABSTRACT FROM AUTHOR]

Background: Biologics are the cornerstone of treatment of patients with moderate‐to‐severe plaque psoriasis and switches between biologics are frequently needed to maintain clinical improvement over time. Objectives: The main purpose of this study was to describe precisely switches between biologics and how their pattern changed over time with the recent availability of new biologic agents. Methods: We included patients receiving a first biologic agent in the Psobioteq multicenter cohort of adults with moderate‐to‐severe psoriasis receiving systemic treatment. We described switches between biologics with chronograms, Sankey and Sunburst diagrams, assessed cumulative incidence of first switch by competing risks survival analysis and reasons for switching. We assessed the factors associated with the type of switch (intra‐class – i.e. within the same therapeutic class ‐ vs. inter‐class) in patients switching from a TNF‐alpha inhibitor using multivariate logistic regression. Results: A total of 2153 patients was included. The cumulative incidence of switches from first biologic was 34% at 3 years. Adalimumab and ustekinumab were the most prescribed biologic agents as first and second lines of treatment. The main reason for switching was loss of efficacy (72%), followed by adverse events (11%). Patients receiving a TNF‐alpha inhibitor before 2016 mostly switched to ustekinumab, whereas those switching in 2016 or after mostly switched to an IL‐17 inhibitor. Patients switching from a first‐line TNF‐alpha inhibitor before 2016 were more likely to switch to another TNF‐alpha inhibitor compared with patients switching since 2018. Patients switching from etanercept were more likely to receive another TNF‐alpha inhibitor rather than another therapeutic class of bDMARD compared with patients switching from adalimumab. Conclusion: This study described the switching patterns of biologic treatments and showed how they changed over time, due to the availability of the new biologic agents primarily IL‐17 inhibitors. [ABSTRACT FROM AUTHOR]

Local malignant potential of basal cell carcinoma (BCC) can lead at advanced stages to the destruction of underlying tissues and significant morbidity. The primary risk factor for progression of advanced basal cell carcinoma (aBCC) is the long duration of the tumour, which results from delay in seeking medical care. To assess the implication of psycho-social factors in the delay before the first medical consultation among patients with aBCC, in order to identify potentially targetable factors enabling earlier diagnosis. Three-step qualitative meta-synthesis: (1) systematic review of the literature; (2) structured qualitative analysis of these documents; (3) construction of a logical model. After screening, 81 articles were included. Self-neglect and denial in patients are roundly put forward as the main obstacles to consultation. We found that avoidance behaviour, mistaken interpretation and banalisation of symptoms, and fear of treatment all played a role. The strongest motivation to seek help comes from the realisation that new symptoms may be dangerous; the role of interpersonal surroundings is highlighted as helpful. Patient delay has multifactorial origins in aBCC, especially self-neglect ranging from denial of tumours to conscious refusal of treatment. [ABSTRACT FROM AUTHOR]

Background Blastic plasmacytoid dendritic cell neoplasm ( BPDCN) is a rare disease characterized by malignant proliferation of a contingent blastic plasmacytoid dendritic cell. This rare entity is recognized mostly by cutaneous spreading, or not having a leukaemic component. The prognosis is very poor. Objectives To study a large cohort of 90 patients with BPDCN, to define additional symptoms to form a correct diagnosis earlier, and to manage such patients accordingly. Methods We retrospectively reviewed BPDCN cases registered in the French Study Group on Cutaneous Lymphoma database between November 1995 and January 2012. Ninety patients were studied. Demographic data, clinical presentation, initial staging and outcome were recorded. Results The group contained 62 male and 28 female patients (sex ratio 2·2). Their ages ranged from 8 to 103 years at the time of diagnosis (mean 67·2 years). Three major different clinical presentations were identified. Sixty-six patients (73%) presented with nodular lesions only, 11 patients (12%) with 'bruise-like' patches and 13 (14%) with disseminated lesions (patches and nodules). Mucosal lesions were seen in five patients (6%). The median survival in patients with BPDCN was 12 months. Conclusions We here distinguish three different clinical presentations of BPDCN. A nodular pattern is a more common feature than the originally reported 'bruise-like' pattern. Despite the fact that BPDCN may initially appear as a localized skin tumour, aggressive management including allogeneic bone marrow transplantation should be considered immediately, as it is currently the only option associated with long-term survival. [ABSTRACT FROM AUTHOR]

Background Primary cutaneous T-cell lymphomas (CTCLs) are malignancies characterized by a clonal T-cell infiltrate involving the skin. CTCLs often show resistance to conventional antineoplastic chemotherapy. Gemcitabine is a pyrimidine analogue which has shown efficacy and a favourable safety profile in solid tumours and haematological malignancies. Objectives  We report a multicentre retrospective study of 23 patients who received gemcitabine for advanced-stage CTCL and emphasize the high incidence of serious unusual adverse events. Methods We collected data from 23 patients with refractory CTCL (14 mycosis fungoides, six Sézary syndrome and three other CTCL). Gemcitabine was given weekly within a 21- or 28-day schedule. Response was evaluated after three and six cycles of chemotherapy. For each patient, all adverse events were recorded. Results Of the 16 patients who received at least three cycles of gemcitabine, 10 achieved a response (62·5%). Only five patients reached the sixth cycle of treatment and four still had a favourable response. Haematological toxicity was recorded in 15 cases with severe grade 3 or 4 neutropenia in seven patients (30%) and six serious infections (26%). Other serious adverse events were observed in six cases (26%): one haemolytic–uraemic syndrome, one severe capillary leak syndrome, one acute heart failure related to cardiac arrhythmia, two bullous and erosive dermatitis, and one recurrent influenza-like syndrome with altered general condition. Conclusions Our study confirms the early efficacy of gemcitabine in advanced-stage CTCL. However, our results contradict the safety profile of gemcitabine previously reported and underline the high incidence of severe complications including visceral and cutaneous involvement. [ABSTRACT FROM AUTHOR]

Abstract: Introduction. – Painful bruising syndrome was described by Gardner and Diamond in 1955. It is marked by spontaneous bruising, without any biological abnormality, affecting young women with pathological mental context. Exegesis. – We report three observations with painful bruising syndrome. In a patient, psychotherapy induced improvement in dermatological and articular manifestations. In other case, placebotherapy made clinical symptoms go away for a prolonged period. Conclusion. – Some etiological hypotheses have been postulated for Gardner and Diamond syndrome. However, published cases speak in favour of psychogenic hypothesis. Somatic and psychological approach must be offered to these patients. [Copyright &y& Elsevier]

Summary A 35-year-old woman with severe fistulizing Crohn's disease presented with pyostomatitis vegetans affecting both the mouth and the vulva. The coalescing pustules transformed within several days into vegetating lesions on areas of inflammation. Microbial assessments revealed no pathogenic agent. Histology showed neutrophilic microabscesses, but no granulomas. Three injections of infliximab and maintenance therapy with methotrexate resulted in rapid and complete regression of both the pyostomatitis vegetans and the Crohn's disease. Infliximab and methotrexate may be a promising treatment for the rare cases of pyostomatitis vegetans associated with Crohn's disease. [ABSTRACT FROM AUTHOR]

Les inhibiteurs de points de contrôles immunitaires (ICI) améliorent significativement la survie de plusieurs cancers mais peuvent provoquer des événements indésirables immunomédiés (immune-related adverse events [irAE]) incluant des arthrites inflammatoires (ICI-IA). Lors d'une progression oncologique, un retraitement par ICI est fréquemment discuté, mais les données chez les patients ayant présenté un épisode d'ICI-IA sont très limitées. L'objectif de notre étude était d'évaluer le risque de récidive et/ou d'aggravation d'ICI-IA à la reprise d'un ICI et les caractéristiques cliniques associées. Il s'agit d'une étude observationnelle multicentrique réalisée dans 4 CHU en France incluant des patients diagnostiqués avec ICI-IA (au moins une synovite ou des symptômes de pseudopolyarthrite rhizomélique [PPR]) ayant débuté un deuxième cycle d'ICI plus de 3 mois après l'arrêt de l'ICI et ayant ≥ 3 mois de suivi post-réadministration de l'ICI. Les données cliniques et biologiques initiales ainsi que l'évolution rhumatologique et oncologique sous retraitement par ICI ont été extraites des dossiers médicaux. Vingt-trois patients ont été inclus et la durée médiane de suivi post-retraitement par ICI était de 8 mois. Le premier épisode d'ICI-IA avait été traité par prednisone (n = 22, 96 %), csDMARD (n = 4,17 %) et anti-IL-6 (n = 1, 4 %). La raison de l'arrêt de l'ICI était l'ICI-IA (n = 8, 35 %), cancer en progression (n = 7, 30 %), autre irAE (n = 4, 17 %) et cancer stable ou en rémission (n = 4, 17 %). Le retraitement par ICI était motivé par une progression oncologique chez tous les patients (n = 22, 96 %) sauf pour un patient présentant une intolérance au traitement de 2e ligne (4 %). Au moment du retraitement par ICI, 18 patients (78 %) ne présentaient aucun symptôme d'ICI-IA, 5 (22 %) avaient une ICI-IA peu symptomatique de grade 1, 11 (48 %) prenaient toujours de la prednisone, 2 (9 %) un csDMARDs et 1 (4 %) un anti-IL-6. Une aggravation ou récidive de l'ICI-IA était retrouvée chez 12 patients (52 %) avec la même présentation clinique que l'histoire articulaire initiale mais de survenue plus précoce (médiane 1 mois). En comparant les patients ayant présenté ou non une poussée d'ICI-IA lors du retraitement par ICI, il n'y avait pas de différence en termes de sexe, d'âge, de classe d'ICI, de présentation d'ICI-IA initiale ni de traitement spécifique de l'ICI-IA. Deux sur 3 (66,7 %) des patients avec facteurs rhumatoïdes et 2/2 (100 %) des patients avec anti-CCP ont présenté une poussée/récidive de l'ICI-IA. Les patients avec cancer du poumon et avec bursite lors de l'ICI-IA initiale étaient plus à risque de présenter une exacerbation. Dans cette première étude sur l'évaluation de la réadministration d'ICI après la survenue d'ICI-IA, environ la moitié des patients ont présenté une récidive ou aggravation de l'ICI-IA. Les patients qui étaient toujours sous traitement rhumatologique spécifique pour contrôler l'ICI-IA au moment de la réintroduction d'ICI semblaient avoir un risque similaire d'exacerbation que ceux qui n'avaient aucun traitement. Certains facteurs de risque cliniques/biologiques semblent être associés au risque de récidive de poussée articulaire inflammatoire. Le retraitement par ICI chez des patients ayant préalablement présenté une ICI-IA au cours d'un premier cycle de traitement par ICI semble sécuritaire et la prise d'immunosuppresseurs au moment du retraitement ne prévient pas la survenue d'une exacerbation. [ABSTRACT FROM AUTHOR]

Summary: Background: Primary cutaneous lymphomas (PCLs) are a heterogeneous group of T‐cell (CTCL) and B‐cell (CBCL) malignancies. Little is known about their epidemiology at initial presentation in Europe and about potential changes over time. Objectives: The aim of this retrospective study was to analyse the frequency of PCLs in the French Cutaneous Lymphoma Registry (GFELC) and to describe the demography of patients. Methods: Patients with a centrally validated diagnosis of primary PCL, diagnosed between 2005 and 2019, were included. Results: The calculated incidence was unprecedently high at 1·06 per 100 000 person‐years. The number of included patients increased yearly. Most PCL subtypes were more frequent in male patients, diagnosed at a median age of 60 years. The relative frequency of rare CTCL remained stable, the proportion of classical mycosis fungoides (MF) decreased, and the frequency of its variants (e.g. folliculotropic MF) increased. Similar patterns were observed for CBCL; for example, the proportion of marginal‐zone CBCL increased over time. Conclusions: Changes in PCL frequencies may be explained by the emergence of new diagnostic criteria and better description of the entities in the most recent PCL classification. Moreover, we propose that an algorithm should be developed to confirm the diagnosis of PCL by central validation of the cases. What is already known about this topic?Primary cutaneous lymphomas (PCLs) are a heterogeneous group of diseases.Little is known about changes over time in their epidemiology and about patients' characteristics and disease stages at the initial presentation. What does this study add?In this population‐based study we present changes over time in PCL epidemiology over a 15‐year period and show an unprecedentedly high incidence in Europe.The relative frequency of rare PCL remained stable over time, while other PCL subtypes were better stratified.This study suggests that the diagnosis of PCL should be evoked more frequently, and delivers up‐to‐date information on patients' characteristics at initial diagnosis in a European population. Linked Comment: Vermeer. Br J Dermatol 2021; 184:993–994. [ABSTRACT FROM AUTHOR]

The 'obesity paradox' suggests that higher body mass index (BMI) is associated with better survival values in metastatic melanoma patients, especially those receiving targeted and immune checkpoint inhibitor therapy. Higher BMI is also associated with higher incidences of treatment-related adverse events (TRAEs). This study assesses whether BMI is associated with survival outcomes and adverse events in metastatic melanoma patients with systemic therapy. This multicentric retrospective study, conducted from 1 March 2013 to 29 April 2019, enrolled adults with unresectable stage III or IV melanoma from the French multicentric prospective cohort-MelBase (NCT02828202). Patients with first-line chemotherapy and targeted and immune therapy were included. Underweight people and those with metastatic mucosal or ocular melanoma were excluded. BMI was categorized using the World Health Organization criteria. Co-primary outcomes included the association between BMI and progression-free survival and overall survival, stratified by treatment type, sex, and age. Secondary endpoints were the association of BMI with overall response and TRAEs. Multivariate analyses were carried out. A total of 1214 patients were analyzed. Their median age was 66.0 years (range, 53-75). Male predominance was observed [ n = 738 (61%)]. Most patients received immune checkpoint inhibitor therapy (63%), followed by targeted therapy (32%), and had stage M1c disease (60.5%). Obese patients represented 22% of the cohort. The median follow-up duration was 13.5 months (range, 6.0-27.5). In the pooled analysis, no positive or negative association between BMI and progression-free survival (P = 0.88)/overall survival (P = 0.25) was observed, regardless of treatment type, sex, and age. These results were nonsignificant in the univariate and multivariate analyses. The objective response rate, according to BMI category, did not differ significantly regardless of age. TRAEs were not associated with BMI. The observed lack of an association between BMI and survival demonstrates that BMI is not a valuable marker of systemic treatment-related outcomes in metastatic melanoma. Future approaches might focus on the whole-body distribution. • Key objective: is high body mass index (BMI) associated with survival outcomes in relation to systemic therapy in patients with metastatic melanoma? • Relevance: in this multicenter, retrospective study of MelBase (French multicentric metastatic melanoma cohort) that included 1214 patients, response to first-line treatment with either chemotherapy, targeted therapy, or immunotherapy was not influenced by BMI. • Knowledge generated: high BMI does not appear to be associated with improved progression-free survival and overall survival in metastatic melanoma patients treated with systemic therapy. Because it does not consider the whole-body composition, others approaches are needed. [ABSTRACT FROM AUTHOR]

The 12-month analysis from Basal Cell Carcinoma Outcomes with LDE225 Treatment (BOLT): a phase II, randomized, double-blind study of sonidegib in patients with advanced basal cell carcinoma. Dear Editor, Basal cell carcinoma (BCC) is the most common human cancer worldwide.1 Although patients are mostly treatable by surgery or radiotherapy, a small proportion progress to having locally advanced (laBCC) or metastatic (mBCC) disease. [Extracted from the article]

Editor Skin manifestations have been increasingly reported in the setting of COVID-19. We retrieved six series, including ours, in which the numbers of both infected patients and patients with skin signs were available.4,6,7,9,10 Cutaneous lesions were observed in 38 patients over 2199 COVID-19 cases. In patients with rashes, severity was found in 64% of cases and death in 2%, while it was respectively found in 5% and 0% patients with chilblains. [Extracted from the article]

Background: Granulomatous foreign body reactions (GFBR) have been reported after injection with almost every soft tissue fillers, more commonly with non‐biodegradable ones. Such granulomatosis is rare but can cause significant discomfort owing to their aesthetic and functional repercussions. Objective: To determine whether immunomodulation with low doses of methotrexate is effective in the treatment of GFBR to filler material. Methods: Clinical case series of four patients with severe, treatment‐resistant GFBR to non‐biodegradable fillers in the Department of Dermatology of Bordeaux University Hospital, Bordeaux, France, successfully treated with oral or subcutaneous methotrexate, 10–15 mg weekly during 6 months. Adverse events were monitored throughout the treatment once weekly the first month then once monthly the remaining 5 months. Results: Four women with a mean age of 73.7 years (66–85 years) and nodularity of the face were included and treated up to 6 months. Histological findings were consistent with GFBR to liquid injectable silicone in 2 cases, polymethylmethacrylate in 1 case and hydroxyethylmethacrylate in the last case. The delay after injection of the filler material was from 17 to 30 years. In one patient, inflammatory lesions followed dental care. After 6 months of treatment with 10–15 mg once weekly, all patients were cleared. Three patients developed a mild hepatic cytolysis (grade 1 or 2). Methotrexate could be maintained in those 3 cases and was discontinuated after 6 months in all cases. Two patients developed recurrence of lesions, 28 and 9 months, respectively, after treatment stops, requiring reintroduction of treatment. The two other patients remained cleared after 6 months of follow‐up. Conclusion: Low doses of methotrexate appear to offer a low‐risk therapeutic alternative in resistant and severe GFBR to fillers. A prospective study with long‐term follow‐up is required to confirm these preliminary observations. [ABSTRACT FROM AUTHOR]

Summary: Background: Mycosis fungoides (MF) and Sézary Syndrome (SS) are the most common cutaneous T‐cell lymphomas. MF/SS is accompanied by considerable morbidity from pain, itching and disfigurement. Aim: To identify factors associated with poorer health‐related quality of life (HRQoL) in patients newly diagnosed with MF/SS. Methods: Patients enrolled into Prospective Cutaneous Lymphoma International Prognostic Index (PROCLIPI; an international observational study in MF/SS) had their HRQoL assessed using the Skindex‐29 questionnaire. Skindex‐29 scores were analysed in relation to patient‐ and disease‐specific characteristics. Results: The study population consisted of 237 patients [60·3% male; median age 60 years, (interquartile range 49–70)], of whom 179 had early MF and 58 had advanced MF/SS. In univariate analysis, HRQoL, as measured by Skindex‐29, was worse in women, SS, late‐stage MF, those with elevated lactate dehydrogenase, alopecia, high modified Severity Weighted Assessment Tool and confluent erythema. Linear regression models only identified female gender (β = 8·61; P = 0·003) and alopecia (β = 9·71, P = 0·02) as independent predictors of worse global HRQoL. Item‐level analysis showed that the severe impairment in symptoms [odds ratio (OR) 2·14, 95% confidence interval (CI) 1·19–3·89] and emotions (OR 1·88, 95% CI 1·09–3·27) subscale scores seen in women was caused by more burning/stinging, pruritus, irritation and greater feelings of depression, shame, embarrassment and annoyance with their diagnosis of MF/SS. Conclusions: HRQoL is significantly more impaired in newly diagnosed women with MF/SS and in those with alopecia. As Skindex‐29 does not include existential questions on cancer, which may cause additional worry and distress, a comprehensive validated cutaneous T‐cell lymphoma‐specific questionnaire is urgently needed to more accurately assess disease‐specific HRQoL in these patients. What's already known about this topic? Cross‐sectional studies of mixed populations of known and newly diagnosed patients with mycosis fungoides (MF)/Sézary syndrome (SS) have shown significant impairment in health‐related quality of life (HRQoL).Previous studies on assessing gender‐specific differences in HRQoL in MF/SS are conflicting.More advanced‐stage disease and pruritus is associated with poorer HRQoL in patients with MF/SS. What does this study add? This is the first prospective study to investigate HRQoL in a homogenous group of newly diagnosed patients with MF/SS.In patients newly diagnosed with MF/SS, HRQoL is worse in women and in those with alopecia and confluent erythema.MF/SS diagnosis has a multidimensional impact on patient HRQoL, including a large burden of cutaneous symptoms, as well as a negative impact on emotional well‐being. Linked Comment:Talpur. Br J Dermatol 2020; 182:541–542. Plain language summary available online [ABSTRACT FROM AUTHOR]

Background: Numerous inclusion and exclusion criteria are involved in phase III moderate to severe psoriasis trials investigating the safety and efficacy of biologics. This questions the generalization of results. Methods: In this cohort study, we applied inclusion/exclusion criteria for phase III trials from original protocols (adalimumab – REVEAL, ustekinumab – PHOENIX, brodalumab – AMAGINE, secukinumab FIXTURE) to all patients enrolled in the PsoBioTeq prospective registry who received a biological agent for the first time between July 2012 and November 2017. We then compared the efficacy, drug survival and occurrence of adverse events between patients who satisfied/did not satisfy the eligibility criteria for these phase III trials. Results: A total of 1267 patients were enrolled, of whom 993 (78.4%) were not eligible for at least one RCT (randomized controlled trial) and 251 (19.1%) did not meet the PASI/PGA severity requirements. Apart from disease severity, the most frequent criteria resulting in exclusion were as follows: non‐plaque psoriasis (12.6%), significant cardiac disease (8.4%), significant liver disease (7.3%), elevated liver enzymes (4.9–9.6%) and personal history of diabetes (9.2%). There was no difference in drug survival between the two groups. The incidence ratio of adverse events was significantly lower in eligible versus non‐eligible patients [0.78 (95% CI 0.62–0.97) (P = 0.03)]. Conclusion: The majority of patients treated with biologics in the PsoBioTeq real‐life registry would not have been eligible for phase III moderate to severe psoriasis trials. Patients not eligible for psoriasis phase III clinical trials have a higher incidence of adverse events. [ABSTRACT FROM AUTHOR]

Presents several correspondence on dermatology. Detection of vascular endothelial growth factor in endothelial cells of dermal vessels; Condition of the patients fingertips; Case of allergic contact dermatitis from betamethasone valerate.

Dear Editor, Chronic spontaneous urticaria (CSU) is an inflammatory disease characterized by spontaneous weals or angio-oedema for more than 6 weeks. International guidelines from the EAACI/GA SP 2 sp LEN/EDF/UNEV were published in 2018,1 but practice remains heterogeneous, especially for CSU refractory to H SB 1 sb antihistamines and regarding states' official drug approval and reimbursement policies. 3 (iii) The working group does not recommend the adjunction of H SB 2 sb antihistamines or montelukast to H SB 1 sb antihistamines in CSU, owing to the lack of demonstrated efficacy. [Extracted from the article]

Dramatic reduction of clindamycin plasma concentration in hidradenitis suppurativa patients treated with the rifampin-clindamycin combination. Dear Editor, Hidradenitis suppurativa (HS) is a painful chronic inflammatory skin disease that impacts quality of life substantially. [Extracted from the article]