[Facial ulcerated nodules revealing primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma]. / Nodules ulcérés du visage révélant un lymphome T cutané épidermotrope CD8+ cytotoxique agressif.

BACKGROUND: Primary cutaneous CD8+ aggressive, epidermotropic, cytotoxic T-cell lymphoma is a rare disease with a poor prognosis. Herein we report a new case, with facial lesions, which was difficult to diagnose. PATIENTS AND METHODS: A 39-year-old woman was hospitalized for ulcerated nodules on the face that had been developing rapidly for 8 weeks. She had visited Djerba, Tunisia, 3 months earlier. No abnormalities were found on previous routine blood tests. Histopathological analysis of a skin biopsy had revealed non-specific lymphocytic infiltrate. Various therapies, including amoxicillin/clavulanic acid, valaciclovir, corticosteroids, colchicine and doxycycline, proved ineffective. Screening of the cutaneous sample for leishmaniasis proved positive using PCR but negative by direct examination and culture. Treatment was initiated with meglumine antimoniate. A further cutaneous biopsy revealed diffuse lymphocytic proliferation and led to a diagnosis of cutaneous CD8+ aggressive, epidermotropic, cytotoxic T-cell lymphoma. A PET scan showed multiple sites of hypermetabolism affecting the face and lymph nodes. Meglumine antimoniate was stopped and the patient experienced complete remission after chemotherapy. CONCLUSION: Ulcerated nodules with acute progression on acral sites are characteristic of cutaneous CD8+ aggressive, epidermotropic, cytotoxic T-cell lymphoma. In our case, the positive result of PCR screening for Leishmania that was ultimately considered a false positive was a confounding factor in the diagnostic process. Regarding therapy, aggressive treatment strategies such as multiagent chemotherapy and hematopoietic stem-cell transplantation are needed due to the rapid progression of the lymphoma.

[Facial skin cancers: Comparison of opinions in French multidisciplinary team meetings]. / Cancers cutanés de la face : avis comparés de réunions de concertation pluridisciplinaire françaises.

BACKGROUND: The aim of our study was to evaluate the diversity, or homogeneity, of recommendations made in multidisciplinary team meetings (MTM) concerning the management of facial skin cancers in France, and to analyze the determinants thereof. PATIENTS AND METHODS: We contacted a panel of dermatology and ENT multidisciplinary teams (MDT) and collected their recommendations made at meetings regarding 3 clinical cases: squamous cell carcinoma in a renal transplant patient with an incomplete excision margin (case 1), locally advanced basal cell carcinoma (case 2), and lentigo maligna (case 3). The responses were analyzed globally and then based on 2 subgroups defined by the presence or absence of a dermatologist in the MTM. The effect of the makeup of the MTM (based on the presence of a dermatologist, a plastic surgeon, an oncologist and an ENT specialist) was evaluated for the main therapeutic proposals. RESULTS: The opinions of the 45 MDMs that responded to the survey were mixed for the three cases as regards important elements such as the indication of surgical revision for case 1, the proposal of an alternative treatment to surgery for case 2, and monitoring arrangements for case 3. Certain proposals were associated with the presence of a dermatologist in the MTM, such as discussion of adaptation of immunosuppressive treatment and details of the surgical margins to be applied for case 1, as well as simple monitoring and details of monitoring arrangements in case 3. CONCLUSION: It is important to maintain dermatologists in MTMs on account of their expertise in all therapeutic areas concerning skin cancers.

[Stage III melanoma: Sentinel node biopsy, completion lymph node dissection and prospects of adjuvant therapy. A French national survey on current and envisaged practices]. / Mélanome au stade ganglionnaire : analyse du ganglion sentinelle, curage ganglionnaire, perspective des traitements adjuvants. Enquête nationale française sur les pratiques actuelles et envisagées.

BACKGROUND: The recent publication of randomized trials investigating the efficacy of adjuvant therapy and completion lymph node dissection at microscopic stage III melanoma calls for a reappraisal of melanoma management from different angles: indications for sentinel lymph node biopsy, indications for completion lymph node dissection in microscopic-stage disease, and adjuvant therapies. Our objective was to evaluate current practices and to question French onco-dermatologists about any changes they envisaged in their practices in the light of recent publications. METHODS: We conducted a national survey among members of the Cutaneous Oncology Group of the French Society of Dermatology in October 2017. RESULTS: Forty French health centers were included, and 53 individual responses were collected. Sentinel lymph node biopsy for melanoma was performed at 75 % of the centers. Before the summer of 2017 and the publication of MSLT-II (proving the absence of any therapeutic benefits for complete lymph node dissection in microscopic stage III melanoma), when a positive sentinel lymph node was diagnosed, immediate completion lymph node dissection was performed at 90 % of the centers. After the publication of MSLT-II, 45 % of the respondents considered stopping this practice. The risk-benefit ratio prompted prescription of nivolumab and of combined dabrafenib+trametinib as adjuvant therapy by respectively 96 % and 79 % of respondents, while the corresponding rates for interferon and ipilimumab were only 21 % and 15 %. CONCLUSION: Early melanoma management stands on the verge of major changes thanks to the arrival of efficient adjuvant therapies and a decrease in immediate completion lymph node dissections for patients with microscopic stage III is also anticipated.

Skin manifestations among GATA2-deficient patients.

GATA2 mutations have been identified in various diseases, such as MonoMAC syndrome, Emberger syndrome, familial myelodysplastic syndrome, acute myeloid leukaemia and dendritic cell, monocyte, B-cell and natural killer-cell deficiency. These syndromes present a wide range of clinical features, dominated by severe infections and haematological disorders such as myelodysplastic syndrome. Up to 70% of patients with GATA2 mutations have dermatological features, mainly genital or extragenital warts, panniculitis or erythema nodosum and lymphoedema. We report three patients presenting with common dermatological and haematological features leading to the diagnosis of GATA2 deficiency, but also with skin manifestations that have not been previously described: gingival hypertrophy, macroglossitis and glossitis and granulomatous lupoid facial lesions. Dermatologists can encounter patients with GATA2 mutations and should recognize this disorder.

[Strontium ranelate-induced DRESS syndrome]. / Syndrome d'hypersensibilité médicamenteuse (DRESS) au ranélate de strontium.

INTRODUCTION: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe form of adverse drug reaction. Strontium ranelate has recently been authorised for postmenopausal osteoporosis. We report a case of strontium ranelate-induced DRESS complicated by linear Ig A dermatosis due to vancomycin. CASE REPORT: A 77-year-old woman with osteoporosis had been treated by strontium ranelate for 4 weeks when she developed a febrile generalized skin rash. Blood tests showed eosinophilia (12.74 × 10(9)/L) and liver damage. A diagnosis of DRESS was made, leading to discontinuation of strontium ranelate and prescription of systemic corticosteroids. Two days later, methicillin-resistant Staphylococcus aureus bacteraemia occurred and treatment with vancomycin was started. The liver dysfunction resolved. After two weeks of antibiotherapy, bullous lesions were noted on the thighs. Skin biopsy results suggested a diagnosis of linear IgA bullous dermatosis. Vancomycin was stopped. Two weeks later, the eruption resolved. The eosinophil count gradually returned to normal after four months of corticosteroid therapy. DISCUSSION: More than 15 cases of DRESS syndrome have been reported in Europe, including 2 deaths related to ranelate strontium, prompting European health authorities to publish a warning concerning the risk of strontium ranelate-induced DRESS. A particular feature of our patient was complication with linear IgA bullous dermatosis caused by vancomycin. In conclusion, it is essential to be aware of the risk of severe cutaneous reaction to strontium ranelate, a new drug used to treat osteoporosis.

The role of some viruses in acute pneumonias in adults.

Virological and bacteriological investigations were performed in 85 patients with acute pneumonias and virus isolation or serological evidence of virus infection were obtained in 37.6% of the cases. Influenza A2 and B viruses were incriminated in 14.1% of the patients; parainfluenza viruses in 7% and adenoviruses in 17.2% of the cases. Coxsackie virus was isolated from one patient's blood, and poliovirus 3 was recovered in 3 cases. In 5 cases associated virus infections were detected.

Importance of associated viral and bacterial infections in the production of acute pneumonias.

Bacteriologic and virologic investigations carried out in a group of 85 patients with acute pneumonia have allowed the determination of the etiologic agent in 52.9% of the cases. Of these, 40% showed combined viral and bacterial infections. Mixovirus infections were frequently associated with staphylococcic ones (7 out of 8 cases) and adenovirus infections with the pneumococcic ones (6 out of 7 cases). The most severe and longdrawn clinical pictures with frequent pleuropulmonary complications (in 22.24% of the cases) had a biphasic evolution in only 6 of the 18 pneumonias of mixed etiology. For their high frequency and their clinical peculiarities, mixed etiology pneumonias might be considered as distinct clinical entities.

Importance of associated viral and bacterial infections in the production of acute pneumonias.

Bacteriologic and virologic investigations carried out in a group of 85 patients with acute pneumonia have allowed the determination of the etiologic agent in 52.9% of the cases. Of these, 40% showed combined viral and bacterial infections. Mixovirus infections were frequently associated with staphylococcic ones (7 out of 8 cases) and adenovirus infections with the pneumococcic ones (6 out of 7 cases). The most severe and longdrawn clinical pictures with frequent pleuropulmonary complications (in 22.24% of the cases) had a biphasic evolution in only 6 of the 18 pneumonias of mixed etiology. For their high frequency and their clinical peculiarities, mixed etiology pneumonias might be considered as distinct clinical entities.