[Spontaneous remission of cutaneous myeloid sarcoma]. / Régression spontanée d'un sarcome myéloïde cutané.

INTRODUCTION: Cutaneous myeloid sarcoma is a rare extramedullary tumour of immature myeloid cells. It is most often associated with acute myeloid leukaemia, and more rarely with myelodysplastic/myeloproliferative disease. PATIENTS AND METHODS: Herein we report a case of cutaneous myeloid sarcoma associated with myelodysplastic/myeloproliferative disease in an 84-year-old man with a diffuse purple papular and nodular rash. The disease course was marked by a spontaneous and total regression for two months. Given the patient's age and general condition, chemotherapy could not be given. DISCUSSION: Cutaneous myeloid sarcoma is rare. It is often associated with acute myeloid leukaemia, and more rarely with myelodysplastic/myeloproliferative disease, but it can occur in isolation. Diagnosis is particularly difficult and is based a consistent body of clinical and histological evidence. Spontaneous regression is very rare and involves immunological mechanisms that are still incompletely understood. Recurrence occurs within a variable time frame and is often associated with transformation to acute myeloid leukaemia if this was not already present at the time of diagnosis. CONCLUSION: Herein we report an extremely rare case of spontaneous regression of cutaneous myeloid sarcoma associated with myelodysplastic/myeloproliferative disease.

[Acitretin-induced capillary leak syndrome: Case report and literature review]. / Syndrome de fuite capillaire observé sous acitrétine : un cas et revue de la littérature.

BACKGROUND: Retinoids are widely used in dermatology. Adverse effects are frequent and require clinical and laboratory monitoring. Herein we report the case of a patient with secondary capillary leak syndrome (SCLS) associated with acitretin. We then present a review of the literature on systemic retinoids and SFCS. PATIENTS AND METHODS: A 57-year-old patient consulted following the onset of severe type I pityriasis rubra pilaris. Treatment was initiated comprising topical corticosteroids combined with acitretin at a dose of 0.5mg/kg/day. On the eighth day, voluminous edema appeared, accompanied by weight gain of 8kg in 48h and hypotension. The laboratory assessment showed hypoalbuminemia and hemoconcentration. Acitretin-induced SCLS was diagnosed based on the triple signs of hemoconcentration, hypoalbuminemia and hypotension, as well as rapid improvement following discontinuation of acitretin. DISCUSSION: We collected 7 published clinical cases between 1981 and 2018, including our own case report. Retinoids were indicated only in severe cutaneous diseases. The mean time to onset of SLCS is 9.8 days, with a return to normal 17 days after discontinuation of retinoids. Capillary leak syndrome is a rare and under-diagnosed clinical-laboratory syndrome that must be recognized in order to avoid potentially fatal inappropriate management. It is a rare adverse effect of retinoids used in dermatology and the pathophysiology remains unclear.

[Carbon dioxide laser treatment of genital intraepithelial neoplasia: A retrospective study]. / Traitement des néoplasies intraépithéliales génitales par laser CO2 : étude rétrospective.

INTRODUCTION: Genital intraepithelial neoplasias (IEN) are precursors of squamous cell carcinoma. The ideal treatment must be efficacious, conservative and well-tolerated. CO2 laser is one of the recommended therapies, but its efficacy and safety have not been extensively studied. PATIENTS AND METHODS: This was a retrospective single-center study. Sixteen patients (8 women and 8 men) treated with CO2 laser for genital IEN, whether high-grade or differentiated, were included. Surgical lateral margins of 3 to 5mm were taken macroscopically during treatment. The CO2 laser session, assessment of therapeutic response and follow-up were undertaken by a dermatologist at the University Hospital of Caen. RESULTS: The mean patient age was 68.1 years and mean follow-up was 52.7 months (5-130). Lesions were isolated in 87.5% of patients. The recurrence rates of genital intraepithelial neoplasia after CO2 laser treatment were 58% for women and 40% for men. No impact on quality of life or on sexual activity was reported in over 90% of patients. CONCLUSION: Whatever the treatment recommended for intraepithelial neoplasia, recurrence is frequent. CO2 laser is an effective and well-tolerated conservative therapeutic option. It enables rapid clinical remission, but as with all currently available treatments, long-term dermatologic follow-up remains necessary.

[Remote dermatological advice: a survey in three French regions]. / Avis dermatologiques à distance : une enquête interrégionale.

BACKGROUND: General practitioners and specialists often seek advice from dermatologists based on digital pictures. This time-consuming activity is currently not valued nor well-established. We therefore conducted a survey to assess the extent and nature of such requests. PATIENTS AND METHODS: A questionnaire was sent to dermatologists working either at university hospitals or in private practice in three regions of France (Loire Atlantique, Rhone Alpes and Normandy). For two months, the dermatologists completed the same questionnaire for each specialized request. The following items were recorded: status of the dermatologist asked to provide a specialized opinion, region in which the request was made, status of the requesting practitioner, mode of request, type of disease, question asked, time taken to answer the request and result of the specialized advice. RESULTS: Thirty dermatologists, including 11 in private practice, participated in this study. We identified 287 requests. The majority of these requests came from general practitioners (30%) and dermatologists working in private practice (29%). These requests were sent by e-mail using a non-secure professional e-mail address in 49% of cases or via smartphone in 29% of cases. The purpose of the requests was either diagnostic/therapeutic (36%) or solely therapeutic (39%). Relevant requests concerned dermatological manifestations in paediatric or cancer patients (23% and 21% respectively), general dermatological diseases (21%) and wound management (14%). Nearly half of the specialized advice was provided in under 5minutes, and in 60% of cases a consultation with a dermatologist was avoided. CONCLUSION: Despite the small number of physicians taking part in the study, this non-exhaustive survey confirms the usefulness of remote dermatological reviews and consultations, and identifies the main intervening physicians involved in this type of procedure. The study also raises potential medico-legal issues regarding the lack of data security covering medical information. There is thus a need for dermatologists to organize their activity in order to respond to such requests without exposing themselves to medico-legal consequences.

[Secondary prevention of myocardial infarction in the Basse-Normandie region]. / Prevention secondaire en post-infarctus en Basse-Normandie.

The objectives of this study were to determine post-infarction drug therapy at discharge from hospital and at one year in the Basse Normandie region (France) and the management of risk factors, to compare them with the practice recommended by the French Society of Cardiology and other recent references. Patients whose medical expenses were exonerated by the Social Security for primary myocardial infarction without a history of angioplasty or of coronary bypass grafting between February and September 2002 were reviewed. The data was researched from the hospital, the patient, the attending physician and the data bases of the Social Security. Four hundred and fifteen patients were included. At discharge from hospital the percentages of prescriptions of recommended drugs were as follows: betablockers 85%, antithrombotics 99%, ACE inhibitors 75%, lipid lowering drugs 90%; the four drug families were associated in 63% of cases. There was no significant difference in prescription between hospital discharge and the twelfth months except with regards to ACE inhibitors (68%) and the association of the four drug groups (54%). The prevalence of smoking, hypertension, diabetes, overweight, obesity and dyslipidaemia were respectively 40, 39, 9, 44, 20 and 81% at the time of infarction. At one year, the prevalence of smoking had fallen significantly to 16%; only 10.3% of patients had uncontrolled hypertension and only 29% had not obtained the recommended therapeutic target for LDL-cholesterol. The authors conclude that this analysis shows an adequation of drug prescription to current recommendations and an improvement in risk factor management which should, however, be pursued.

[Sturge-Weber-Krabbe syndrome. A severe form in a monozygote female twin]. / Syndrome de Sturge-Weber-Krabbe. Forme grave chez une jumelle monozygote.

We report a severe case of Sturge-Weber syndrome in one of two monozygotic twins. This syndrome included a facial portwine stain over the trigeminal ophthalmic V1 area, and ipsilateral vascular anomalies of the eye and of the pia mater. CT scans and MRI were very informative. The cerebral regional blood flow, studied with SPECT, showed a paradoxically high rate.

[Acral erythema after allogeneic graft of the bone marrow]. / Erythème acral après greffe de moelle osseuse allogénique.

Acral erythema (AE) is a painful, erythematous bullous eruption of the palms and soles which is chemotherapy-induced. To the numerous chemotherapies which induce AE we must add, perhaps, a new drug, Vépéside. AE is followed by graft-versus-host-disease in all patients receiving bone marrow transplantation. AE and early GVH disease being very similar, we discuss the differential diagnosis which can be very difficult.

Keratoderma climactericum (Haxthausen's disease): clinical signs, laboratory findings and etretinate treatment in 10 patients.

10 cases of keratoderma climactericum are reported. This keratosis of the palms and soles appears late in women of menopausal age. The keratotic lesions first develop at the plantar pressure points, making walking troublesome. Involvement of the hands remains discrete. Examination for contact allergy, fungal tests, vitamin A serum levels, and sex hormones were negative or normal in all the 10 patients. Microscopy revealed a lichenified eczema with evidence of mechanical irritation. Etretinate (0.78 mg/kg/day) brought about partial or total remission of the hyperkeratosis. Pain on walking disappeared in all the patients.

Photo-induction of lesions in a patient with pemphigus erythematosus.

Ultraviolet light applied to the normal appearing skin of a patient with pemphigus erythematosus produced clinical and histological changes, typical of the disease, in the irradiated skin. The action spectrum, studied with monochromatic light, was in the range of UVB.

[Acyclovir treatment of eczema herpeticum in Darier's disease]. / Traitement par acyclovir d'un eczema herpeticum compliquant une maladie de Darier.

This paper describes two episodes of eczema herpeticum occurring in a woman with Darier's disease. During the first episode, the vesicular eruption represented a primary herpetic infection because there were no IgM herpes antibodies. The second attack suggested a recurrent infection. Herpes simplex, virus type I was isolated from the lesions in both cases.