[Cutaneous manifestation of lymphoid variant of hypereosinophilic syndrome: One case]. / Un cas de manifestation cutanée d'un syndrome hyperéosinophilique lymphoïde.

INTRODUCTION: Lymphoid hypereosinophilic syndrome (HES) is a reactive HES, related to the presence of an abnormal circulating T cell clone. Cutaneous manifestations are frequent and sometimes inaugural, however few studies describe them specifically. CASE REPORT: We report the case of a 63-year old patient, in good general condition, with no previous history and taking no treatment, who was being followed for non-specific skin lesions. Blood and skin examinations showed hypereosinophilia, the presence of an aberrant CD3-CD4+ phenotype and a positive T-clonality test. There was no differential diagnosis or argument for a systemic lymphoma. CONCLUSION: Cutaneous manifestations of lymphoid HES are variable, non-specific, and may differ according to lymphocyte phenotype. The discovery of SHE requires an extension workup and the risk of evolution towards a systemic lymphoma justifies a close surveillance. Treatment is adapted to the severity of the symptoms.

Lymphomatoid papulosis types D and E: a multicentre series of the French Cutaneous Lymphomas Study Group.

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.

Relevance of body mass index as a predictor of systemic therapy outcomes in metastatic melanoma: analysis of the MelBase French cohort data☆.

BACKGROUND: 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. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Update of survival and cost of metastatic melanoma with new drugs: Estimations from the MelBase cohort.

PURPOSE: Since 2011, significant progress was observed in metastatic melanoma (MM), with the commercialisation of seven immunotherapies or targeted therapies, which showed significant improvement in survival. In France, in 2004, the cost of MM was estimated at €1634 per patient; this cost has not been re-estimated since. This study provided an update on survival and cost in real-life clinical practice. METHODS: Clinical and economic data (treatments, hospitalisations, radiotherapy sessions, visits, imaging and biological exams) were extracted from the prospective MelBase cohort, collecting individual data in 955 patients in 26 hospitals, from diagnosis of metastatic disease until death. Survival was estimated by the Kaplan-Meier method. Costs were calculated from the health insurance perspective using French tariffs. For live patients, survival and costs were extrapolated using a multistate model, describing the 5-year course of the disease according to patient prognostic factors and number of treatment lines. RESULTS: Since the availability of new drugs, the mean survival time of MM patients has increased to 23.6 months (95%confidence interval [CI] :21.2;26.6), with 58% of patients receiving a second line of treatment. Mean management costs increased to €269,682 (95%CI:244,196;304,916) per patient. Drugs accounted for 80% of the total cost. CONCLUSION: This study is the first that evaluated the impact of immunotherapies and targeted therapies both on survival and cost in real-life conditions. Alongside the introduction of breakthrough therapies in the first and subsequent lines, MM has been associated with a significant increase in survival but also in costs, raising the question of financial sustainability.

Kaposi's sarcoma and pregnancy: case report and literature review.

We report the observation of cutaneous Kaposi's sarcoma (KS) worsening during the second trimester of pregnancy in 2 African women. Both patients were tested seronegative for HIV. They had no complication during their pregnancy, and no evidence of extracutaneous disease was found, allowing therapeutic abstention. They gave birth to healthy children showing no clinical evidence of human herpesvirus 8 (HHV-8) infection. Based on these observations and on the review of the literature, we discuss the risk of vertical transmission of HHV-8 as well as the impact of pregnancy on KS.

[Stevens-Johnson syndrome concurrent with primary Epstein-Barr virus infection]. / Syndrome de Stevens-Johnson au cours d'une primo-infection par le virus d'Epstein-Barr.

BACKGROUND: Primary EBV infection mainly affects children and young adults. Most patients present the characteristic triad of fever, pharyngitis and lymphadenopathy. Symptoms may include a morbilliform eruption, occasionally induced by amoxicillin. We report a case of Stevens-Johnson syndrome concurrent with EBV infection. CASE REPORT: A 17-year-old boy consulted for an eruption suggestive of Stevens-Johnson syndrome, which was supported by the histopathology results. The patient had taken no medication during the previous weeks. Laboratory examinations showed atypical activated T lymphocytes. Serological tests and PCR results confirmed the diagnosis of primary EBV infection. The outcome was spontaneously favorable with only symptomatic treatment being required. DISCUSSION: Stevens-Johnson syndrome is characterized by "target" lesions and profuse mucous membrane involvement. Stevens-Johnson syndrome is frequently drug-induced, being due less frequently to infections. Stevens-Johnson syndrome is very rarely a manifestation of infectious mononucleosis, with only one case being reported in the literature. When confronted with Stevens-Johnson syndrome without any imputable medication, complete screening for infection should be performed, in particular for primary EBV infection.

Focal rituximab-induced edematous reaction at primary cutaneous follicle center lymphoma lesions: case report and literature review.

Primary cutaneous follicle center lymphoma (PCFCL) is the most common cutaneous B cell lymphoma. It is most often indolent and responds well to rituximab. We present a case of transient rituximab-induced edematous lesions located exclusively on tumor papules in a patient treated for PCFCL. Based on this observation and on a review of the literature, we discuss the mechanism of this edematous reaction which does not seem to be allergic. Indeed, this focal reaction observed solely during the first infusion of rituximab is more likely linked with local cytokine release induced by B cell lysis in the skin. This reaction is neither unusual nor severe and should not lead to an interruption of rituximab.