Barriers and suggestions for improving the implementation of guidelines in the systemic treatment of moderate to severe psoriasis: A literature review.

BACKGROUND: Despite the availability of a wide range of therapies for the systemic treatment of moderate to severe psoriasis, many psoriasis patients do not receive adequate treatment, suggesting that guidelines may not be correctly applied by physicians. OBJECTIVES: The aim of this study was to analyze data on physicians' implementation of, and reasons for noncompliance with, guidelines for the systemic treatment of moderate to severe psoriasis. METHODS: We conducted a systematic literature review according to the Preferred Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We searched the PubMed and Embase databases for studies on guideline adherence in the systemic treatment of moderate to severe psoriasis. All eligible articles were retrieved in full text and the relevant references of retrieved articles were included. RESULTS: A total of 20 studies were selected. Four studies investigated knowledge of the guidelines, six studies examined their application, and five studies focused on the various barriers to implementation. Finally, five studies discussed ways to improve implementation. Several studies on the quality of psoriasis care have revealed discrepancies between the reality and the optimal care described in national and international guidelines. CONCLUSION: Various barriers to implementation of recommendations exist, such as economic barriers, lack of dermatologic orientation towards, lack of knowledge of recommendations by other specialists, lack of applicability, and country- and practice-specific features (e.g., different benefit/risk ratios, different reimbursement rates and conditions). This review can help the everyday practitioner to better understand these barriers, which will have a direct impact on improving the quality of life of psoriasis patients.

Characteristics of atopic dermatitis patients using complementary and alternative medicine: A literature review.

BACKGROUND: Atopic dermatitis (AD) is a chronic inflammatory skin disease. Due to the burden of the disease, some patients try complementary and alternative medicine (CAM). OBJECTIVE: To identify characteristics associated with CAM use in children and adults with AD. METHODS: We conducted a literature review in accordance with the PRISMA international guidelines for literature reviews and meta-analyses. A systematic search was performed in the PubMed database. Qualitative and quantitative analyses using a χ2 test were performed to compare characteristics between CAM users and non-users. A p-value of <0.05 was considered statistically significant. RESULTS: Out of 514 articles retrieved, 12 studies were included, giving a total of 2240 patients. Our statistical analysis identified an association between CAM use and rhino-conjunctivitis (p = 0.015 in children, p = 0.041 in adults), topical corticosteroid use (p = 0.042 in children, p = 0.008 in adults), and daily application of moisturizing cream (p = 0.002 in children, p < 0.001 in adults). Gender did not affect the decision to use CAM (p > 0.05). In studies, a higher number of affected eczema sites (p < 0.001), prior use of more than two conventional treatments (p = 0.047), and food avoidance diets (p = 0.016) were predictive of CAM use in children. In adults, a younger age (p < 0.05), higher education level (p = 0.043), and lower age at AD onset (p = 0.004) were related to CAM use. DISCUSSION: To our knowledge, this is the first literature review focusing on socio-demographic and disease determinants related to CAM use among AD patients. The lack of homogeneity in measuring tools makes it difficult to compare and synthesize the studies.

Efficacy of dupilumab in chronic prurigo and chronic idiopathic pruritus: a systematic review of current evidence and analysis of response predictors.

Dupilumab has demonstrated a great reduction in chronic pruritus that is the hallmark of atopic dermatitis (AD). Underscoring relevant pathogenesis similarities emerging from AD, chronic idiopathic pruritus (CIP) and chronic prurigo (CP), several authors suggested the beneficial role of dupilumab in these conditions. The evidence on this subject is limited with no precise data available. In this study, we carried out a systematic literature review in order to evaluate the efficacy of dupilumab on both pruritus and skin manifestations in the two largest retrospective cohorts of patients with CP and CIP and tried to identify potential response predictors. Electronic searches were conducted on 4 databases. Our primary outcome was the improvement in pruritus measured by a reduction in the patient's reported numerical rating scale of itch (NRSI) by >4. Secondary outcomes included the proportion of patients with a complete response at the end of treatment, reduction in the number of lesions by the Investigator Global Assessment (IGA), improvement in numerical rating scale of sleep (NRSS), improvement in quality of life measured by the Dermatology Life Quality Index (DLQI), time until patient perceived any improvement (Time-First) and time until the patient-reported absence of pruritus (Time-Final). Descriptive statistics were calculated for each demographic and clinical variable. Univariate logistic regression analyses were conducted to explore the association between response to dupilumab and potential predictive factors. We included 25 articles in the analysis, counting a total of 153 patients. Based on CP patients' cohort (n = 132), the mean NRSI at baseline was 8.79 ± 0.86 and the NRSI final was 2.32 ± 1.27. The mean time to first improvement was 5.18 ± 3.13 weeks, while the time to complete improvement of pruritus (Time-final) was 13.6 ± 12.0 weeks. Ninety patients out of 109 (83%) noticed an improvement in pruritus before 4 weeks of dupilumab therapy. At the end of treatment, 18 patients out of 126 (14%) had a complete remission of pruritus and 110 patients out of 123 (89%) had a reduction of NRSI >4. The reduction in NRSI was significantly greater in patients improving before 4 weeks of treatment (6.57 ± 1.71) compared with patients improving in more than 4 weeks (5.49 ± 1.39, P < 0.001). Patients with history of AD and those who have been previously treated with cyclosporine or methotrexate had a significantly lower reduction in NRSI (e.g. 6.05 ± 1.34 vs. 7.08 ± 1.90, P < 0.01 for nonassociated AD patients). Based on CIP patient's cohort (n = 21), the mean NRSI at baseline was 8.33 ± 0.80 and the NRSI final was 0.95 ± 0.59. The mean time to first improvement was 2 ± 0 weeks, while the time to complete improvement (Time-final) was 14.6 ± 10 weeks. At the end of treatment, 3 patients out of 21 (14%) had a complete remission of pruritus and 100% of patients had a reduction of NRSI >4. No serious treatment-emergent adverse events were reported. The most common adverse event was mild conjunctivitis (13 cases). We highlight the importance of one early sign of improvement as a predictor of the future response to dupilumab: the improvement before 4 weeks of treatment that leads significantly to a greater final reduction in NRSI. Furthermore, patients with the presence or history of atopy appear to be less responsive to dupilumab than nonatopic patients and develop more side effects, in particular conjunctivitis.

Dermatological adverse effects of anti-glaucoma eye drops: a review.

Many patients are treated for glaucoma. Like other drugs, anti-glaucoma eye drops may induce dermatological adverse effects. We aim to review the dermatological adverse effects secondary to the active agents in anti-glaucoma eye drops through a literature review. In January 2020, we queried PubMed using the following MeSH terms: glaucoma/drug therapy or glaucoma, open angle/drug therapy cross-referenced with parasympathomimetics/adverse effects or adrenergic agonists/adverse effects or carbonic anhydrase inhibitors/adverse effects or prostaglandins F, synthetic/adverse effects or adrenergic beta antagonists/adverse effects or ophthalmic solutions/adverse effects. The initial search identified 1128 studies, of which 49 were excluded for being in a foreign language, 15 for not involving eye drops, 968 for not focusing on adverse dermatological effects, and 11 for insufficient documentation or redundancy. After adding 38 linked studies, we finally analyzed 123 studies. The ocular and periocular dermatological adverse effects of eye drops are contact dermatitis, hyperpigmentation, prostaglandin analog periorbitopathy, mucous membrane pemphigoid, eyelash depigmentation, skin hypertrichosis, and rare cases of melanoma and skin depigmentation. The reported distant dermatological adverse effects are psoriasis, excessive sweating, lichen planus, alopecia, toxic epidermal necrolysis, erythema multiforme, erythroderma, subacute cutaneous lupus erythematosus, nail pigmentation, and bullous pemphigoid. Most of the cutaneous adverse effects of anti-glaucoma eye drops are ocular and periocular and induced by prostaglandin analogs. Distant adverse effects are rare and sometimes questionable but should be kept in mind, especially mucous membrane pemphigoid, which could lead to blindness. The role of preservatives, such as benzalkonium chloride, should also be considered.

Effectiveness and safety of dupilumab in the treatment of atopic dermatitis in children (6-11 years): data from a French multicentre retrospective cohort in daily practice.

BACKGROUND: Dupilumab is the first biotherapy available for the treatment of moderate-to-severe childhood atopic dermatitis (AD). OBJECTIVE: The aim of this study was to evaluate the effectiveness and safety of dupilumab in daily practice. METHODS: Patients aged 6-11, who had received a first dose of dupilumab, were included in this multicentre retrospective cohort study. The primary endpoint was change in SCORAD after 3 months of treatment. Secondary endpoints were change in IGA score at 3 months, proportion of patients with SCORAD50 and SCORAD75, description of adverse events and proportion of children in our cohort who would be excluded from pivotal phase 3 clinical trial. RESULTS: Eighty patients were included. After 3 months of treatment, there was a significant decrease in SCORAD (mean: 21.8 ± 13.8 vs 53.9 ± 18.5; P < 0.0001) and IGA (1.3 ± 0.8 vs 3.5 ± 0.7; P < 0.0001). Conjunctivitis was observed in 11.3% (n = 9/80); three patients experienced dupilumab facial redness (DFR); 17.5% (n = 14/80) reported injection site reactions; 6.3% (n = 5/80) discontinued treatment. 61.2% (n = 49/80) children were ineligible in the phase 3 trial. LIMITATIONS: There is no control group. Because it was a real life study based on information from patient medical records in a French multicentre cohort, we cannot rule out the presence of reporting bias generated by the use of patient reported characteristics and missing information. CONCLUSION: These real-life data confirm the efficacy and safety of dupilumab in children with moderate to severe AD extended to dyshidrosis and atopic prurigo, but it also revealed a lower frequency of DFR and conjunctivitis. However, administration in injectable form may be a barrier in this age group.

Children with psoriasis and COVID-19: factors associated with an unfavourable COVID-19 course, and the impact of infection on disease progression (Chi-PsoCov registry).

BACKGROUND: The COVID-19 pandemic has raised questions regarding the management of chronic skin diseases, especially in patients on systemic treatments. Data concerning the use of biologics in adults with psoriasis are reassuring, but data specific to children are missing. Moreover, COVID-19 could impact the course of psoriasis in children. OBJECTIVES: The aim of this study was therefore to assess the impact of COVID-19 on the psoriasis of children, and the severity of the infection in relation to systemic treatments. METHODS: We set up an international registry of paediatric psoriasis patients. Children were included if they were under 18 years of age, had a history of psoriasis, or developed it within 1 month of COVID-19 and had COVID-19 with or without symptoms. RESULTS: One hundred and twenty episodes of COVID-19 in 117 children (mean age: 12.4 years) were reported. The main clinical form of psoriasis was plaque type (69.4%). Most children were without systemic treatment (54.2%); 33 (28.3%) were on biologic therapies, and 24 (20%) on non-biologic systemic drugs. COVID-19 was confirmed in 106 children (88.3%) and 3 children had two COVID-19 infections each. COVID-19 was symptomatic for 75 children (62.5%) with a mean duration of 6.5 days, significantly longer for children on non-biologic systemic treatments (P = 0.02) and without systemic treatment (P = 0.006) when compared with children on biologics. The six children who required hospitalization were more frequently under non-biologic systemic treatment when compared with the other children (P = 0.01), and particularly under methotrexate (P = 0.03). After COVID-19, the psoriasis worsened in 17 cases (15.2%). Nine children (8%) developed a psoriasis in the month following COVID-19, mainly a guttate form (P = 0.01). DISCUSSION: Biologics appear to be safe with no increased risk of severe form of COVID-19 in children with psoriasis. COVID-19 was responsible for the development of psoriasis or the worsening of a known psoriasis for some children.

Characteristics of children and adolescents with atopic dermatitis who attended therapeutic patient education.

BACKGROUND: Atopic dermatitis (AD) is a chronic, relapsing, inflammatory skin disease. Therapeutic patient education (TPE) has been demonstrated to be effective in AD in reducing disease severity and improving coping and quality of life. OBJECTIVES: To describe the sociodemographic and clinical characteristics of children and adolescents with AD who had attended TPE sessions, as well as the characteristics of their parents, and compare them with those who did not attend TPE. METHODS: Parents of children with AD aged 6-17 years old were recruited from a representative sample of the French population contacted by e-mail. Sociodemographic data and clinical information were collected in patients and parents. Clinical severity was assessed by parents using a proxy version of the Patient-Oriented Eczema Measure (POEM). Attendance to TPE sessions was assessed by the following question 'did your child or one or both parents attended TPE for AD?'. Also, the number of sessions was recorded. Determinants of TPE attendance were evaluated by univariable and multivariable analyses. RESULTS: Data were collected on 1063 parents and children with AD. A total of 131 (12.3%) children and/or parents attended TPE sessions. Most of them attended 2-5 TPE sessions. In that group, there were 85 boys (64.9%), and severity evaluated by POEM was mild in 29.8%, moderate in 52.7% and severe in 17.6% of patients. In the multivariable model, attending TPE sessions was significantly associated with sex of the child (boy vs. girl), consultation with a dermatologist or a paediatrician, high clinical severity and presence of AD in parents. CONCLUSIONS: Despite recommendations, the use of TPE in children with AD is still low in France. There is a need for implementing such programmes in the management of the disease, in particular when the disease is severe.

Ivermectin safety in infants and children under 15 kg treated for scabies: a multicentric observational study.

BACKGROUND: Scabies is a frequent condition seen in infants and children. Only topical treatments have been approved in infants, but some of them are poorly tolerated. Oral ivermectin is approved for the treatment of scabies in several countries, but its use in infants and children weighing < 15 kg is off label. OBJECTIVES: To assess the safety of ivermectin in infants and young children, and to collect data on ivermectin efficacy in these age groups. METHODS: This study was performed in the dermatology and paediatric dermatology departments of 28 French centres between July 2012 and November 2015. Physicians treating an infant or child weighing < 15 kg for scabies with oral ivermectin were asked to send back a completed standardized and anonymous questionnaire, and the data were analysed. RESULTS: Data were collected on 170 infants and children aged 1-64 months, with a body weight of 4-14·5 kg, who were treated with oral ivermectin. The mean dose received was 223 µg kg-1 and 89% of the patients received a systematic second dose. Concomitant topical treatment was administered to 73% of patients. Adverse events were reported in seven patients (4%) and were not severe. At the follow-up visit, 139 (85%) patients had achieved healing. Factors significantly associated with healing were an ivermectin dose > 200 µg kg-1 (P < 0·001), and a delay between those two doses of < 10 days (P = 0·025). CONCLUSIONS: Our findings suggest the safety and efficacy of ivermectin for the treatment of scabies in infants and young children. What's already known about this topic? Scabies is a frequent condition in small children and infants, but the therapeutic options are limited. Ivermectin has been approved for the treatment of scabies in adults and children > 15 kg, but its use is off-label in infants and children weighing < 15 kg. Safety data on the use of ivermectin in children weighing < 15 kg are limited. What does this study add? Of 170 infants and children weighing < 15 kg who were treated for scabies with oral ivermectin, there were only seven reported mild adverse events and no serious ones. Our results show that ivermectin is effective in treating scabies in 85% of patients. Efficacy is higher when the received dose exceeds 200 µg kg-1 and when the delay between the two doses is < 10 days. Respond to this article.

[Fatal interstitial lung disease and pneumocystis during dermatomyositis associated with anti-MDA5 antibodies]. / Dermatomyosite à anticorps anti-MDA-5 compliquée d'une pneumopathie interstitielle et d'une pneumocystose d'évolution fatale.

BACKGROUND: Dermatomyositis (DM) in an auto-immune inflammatory myopathy with skin lesions, and, occasionally, organ involvement. Herein, we report a case of DM during anti-MDA5 antibody therapy associated with interstitial lung disease (ILD) and pneumocystosis. PATIENTS AND METHODS: A 64-year-old woman was hospitalized for impairment of her general health and skin lesions. Dermatological examination revealed classic signs of DM associated with hyperkeratotic papules on the palm creases. This led us to suspect DM with anti-MDA5 antibodies, which was subsequently confirmed by immunologic tests. We also noted dysphonia, exertional dyspnea and proximal muscles weakness. Despite early corticosteroid therapy, combined later with azathioprine, the patient's dyspnoea worsened; one month later, sudden pulmonary decompensation resulted in her admission to intensive care. A chest scan showed evidence of ILD and infectious signs, and the bronchoalveolar lavage was positive for Pneumocystisjiroveci. Despite treatment of this opportunist infection with cotrimoxazole and intensified immunosuppression, the patient died in intensive care. DISCUSSION: Anti-MDA5 antibodies are associated with a specific clinical phenotype and a high degree of risk that should alert the dermatologist to the high likelihood of ILD having a poor prognosis. Associated clinical signs are erythematous, hyperkeratotic or ulcerated papules on the palm creases, as well as fingertip or periungual ulcerations or digital necrosis. This situation is associated with a high risk of pneumocystosis. However, no recommendations concerning prophylaxis are currently available.

[Epidermolysis bullosa acquisita with Brunsting-Perry type pemphigoid: Diagnostic and therapeutic difficulties]. / Épidermolyse bulleuse acquise à type de pemphigoïde de Brunsting-Perry : difficultés diagnostiques et thérapeutiques.

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a rare auto-immune blistering disease. We report a case of Brunsting-Perry pemphigoid diagnosed by immunoelectron microscopy (IEM). PATIENTS AND METHODS: A 46-year-old man presented very pruriginous vesicles on the face and neck present for 6 years and which were difficult to diagnose and treat. The appearance of atrophic scars and milium cycts evoked EBA, which was confirmed at IEM. Due to limited involvement of the face and the neck, we conclude on EBA of the Brunsting-Perry pemphigoid variant. Treatment with dapsone produced a favorable outcome. DISCUSSION: Diagnosis of EBA is often difficult. In a case review, Asfour et al. collated 60 cases of Brunsting-Perry pemphigoid. These patients had either anti-collagen VII or anti-BP180 and anti-BP230 antibodies. IEM showed cleavage either under the lamina densa or within the lamina lucida, suggesting that Brunsting-Perry pemphigoid is a subtype of EBA or bullous pemphigoid (BP), depending on the paraclinical elements, and localized to the head and neck. The majority of EBA-like cases required systemic therapy, whereas in the presence of BP antibodies, topical corticosteroids were effective. CONCLUSION: We report a case of EBA of the Brunsting-Perry pemphigoid type, diagnosed by IEM after 6 years of progression. We highlight the diagnostic and nosological difficulties of Brunsting-Perry pemphigoid. Classification of this dermatosis as a subtype of EBA or BP may enable effective adaptation of therapeutic management, which has not as yet been coded.

[Off-label drugs in childhood psoriasis]. / Prescriptions hors AMM (autorisation de mise sur le marché) dans le psoriasis de l'enfant.

INTRODUCTION: Psoriasis affects 0.5% of children in Europe, with moderate to severe clinical forms in 15-35% of cases warranting the use of systemic treatments. Few treatments are licensed for childhood psoriasis. In this study, we analyzed the frequency of such prescriptions. MATERIALS AND METHODS: Our study was based on 3 retrospective cohort trials conducted in France between 2012 and 2018: χ-Psocar (313 children with psoriasis seen in hospitals), PsoLib (207 children seen in a private practice), and BiPe (134 children on biotherapies). Our evaluation was centered on off-label use. To avoid duplicates between cohorts, analysis focused on each cohort independently. RESULTS: In the χ-Psocar study, in 34.8% of cases, use of at least one off-label treatment, mainly topical vitamin D (36.0%), and systemic treatments (methotrexate and cyclosporine) was noted, on account of either the clinical type of psoriasis (13.7%) or patient age (24.6%). In the PsoLib study, in 41.5% of cases, at least one off-label treatment was noted, mainly combined calcipotriol-betamethasone (24.2%), ciclopirox shampoo (7.2%) and systemic treatments (n=20). The main reason was patient age (41.5%). In the BiPe study, in 97.0% of cases, at least one off-label treatment was noted. These prescriptions mainly concerned a combination of calcipotriol-betamethasone (68.7%) and tacrolimus (11.2%) along with systemic treatment comprising methotrexate, cyclosporin, methoxsalen or apremilast (n=125), but also biotherapies (n=85). The biotherapies were used off-label since at that time they had not yet been granted marketing authorisation. DISCUSSION: This study focused on 3 cohorts of children with psoriasis seen either in private practice or in a hospital setting, and it involved all types of treatment. Off-label prescriptions ranged from one-third to almost 100% of the children, depending on the individual cohorts. The prescribed drugs were topical treatments, conventional systemic drugs and biotherapies. Off-label prescription is not strictly prohibited in France provided it is within a well-defined regulatory framework. Where there is a rich bibliography, confident recommendations may be made. Unfortunately, in childhood psoriasis, the literature and recommendations are very limited, leaving prescribers with considerable individual responsibilities. Review of the license concerning children with psoriasis, a push to conduct therapeutic studies and the drafting of recommendations all appear necessary.

Dermatological manifestations in cardiofaciocutaneous syndrome: a prospective multicentric study of 45 mutation-positive patients.

BACKGROUND: Data on dermatological manifestations of cardiofaciocutaneous syndrome (CFCS) remain heterogeneous and almost without expert dermatological classification. OBJECTIVES: To describe the dermatological manifestations of CFCS; to compare them with the literature findings; to assess those discriminating CFCS from other RASopathies, including Noonan syndrome (NS) and Costello syndrome (CS); and to test for dermatological phenotype-genotype correlations. METHODS: We performed a 4-year, large, prospective, multicentric, collaborative dermatological and genetic study. RESULTS: Forty-five patients were enrolled. Hair abnormalities were ubiquitous, including scarcity or absence of eyebrows and wavy or curly hair in 73% and 69% of patients, respectively. Keratosis pilaris (KP), ulerythema ophryogenes (UO), palmoplantar hyperkeratosis (PPHK) and multiple melanocytic naevi (MMN; over 50 naevi) were noted in 82%, 44%, 27% and 29% of patients, respectively. Scarcity or absence of eyebrows, association of UO and PPHK, diffuse KP and MMN best differentiated CFCS from NS and CS. Oral acitretin may be highly beneficial for therapeutic management of PPHK, whereas treatment of UO by topical sirolimus 1% failed. No significant dermatological phenotype-genotype correlation was determined. CONCLUSIONS: A thorough knowledge of CFCS skin manifestations would help in making a positive diagnosis and differentiating CFCS from CS and NS.

Dermatological manifestations in Noonan syndrome: a prospective multicentric study of 129 patients positive for mutation.

BACKGROUND: Data on dermatological manifestations of Noonan syndrome (NS) remain heterogeneous and are based on limited dermatological expertise. OBJECTIVES: To describe the dermatological manifestations of NS, compare them with the literature findings, and test for dermatological phenotype-genotype correlations with or without the presence of PTPN11 mutations. METHODS: We performed a large 4-year, prospective, multicentric, collaborative dermatological and genetic study. RESULTS: Overall, 129 patients with NS were enrolled, including 65 patients with PTPN11-NS, 34 patients with PTPN11-NS with multiple lentigines (NSML), and 30 patients with NS who had a mutation other than PTPN11. Easy bruising was the most frequent dermatological finding in PTPN11-NS, present in 53·8% of patients. Multiple lentigines and café-au-lait macules (n ≥ 3) were present in 94% and 80% of cases of NSML linked to specific mutations of PTPN11, respectively. Atypical forms of NSML could be associated with NS with RAF1 or NRAS mutations. In univariate analysis, patients without a PTPN11 mutation showed (i) a significantly higher frequency of keratinization disorders (P = 0·001), including keratosis pilaris (P = 0·005), ulerythema ophryogenes (P = 0·0001) and palmar and/or plantar hyperkeratosis (P = 0·06, trend association), and (ii) a significantly higher frequency of scarce scalp hair (P = 0·035) and scarce or absent eyelashes (P = 0·06, trend association) than those with PTPN11 mutations. CONCLUSIONS: The cutaneous phenotype of NS with a PTPN11 mutation is generally mild and nonspecific, whereas the absence of a PTPN11 mutation is associated with a high frequency of keratinization disorders and hair abnormalities.

[Extra-nodal NK/T-cell lymphoma, nasal-type, revealed by cutaneous and ocular involvement]. / Atteintes cutanée et oculaire révélatrices d'un lymphome T/NK extra-nodal de type nasal.

BACKGROUND: Extra-nodal NK/T-cell lymphoma (ENKTL) is a form of highly malignant non-Hodgkin's lymphoma. There are two types: nasal forms primarily affecting the oropharyngeal sphere and so-called nasal-type extra-nasal forms in which primary skin involvement is the most common feature enabling diagnosis. Herein, we report a case of systemic nasal-type ENKTL (ENKTL-NT) that was diagnosed based on skin involvement associated with ocular involvement. PATIENTS AND METHODS: A 67-year-old female patient, without immunodepression, was admitted to the dermatology department for a worsening inflammatory scaly patch of skin on her right calf. Secondarily, further lesions appeared on her body as well as a generalized macropapular rash and sores. These were associated with fever spikes, as well as ophthalmoplegia and edema, preventing her from opening her right eyelid. Tests for infectious, autoimmune and inflammatory disorders were negative. A cerebro-orbital scan revealed infiltration and contrast enhancement of the right periocular fat without any mass effect or cerebral extension. A positron emission tomography (PET) scan revealed multiple hypermetabolic skin lesions. Histological analyses indicated dermal-hypodermal lymphomatous tumor proliferation, and immunohistochemical analyses revealed lymphocytes expressing NK-cell markers (strong CD56+ expression), cytotoxic markers (granzyme B and TIA-1), and the presence of Epstein Barr virus (EBV) in the tumor cells. The patient was diagnosed with systemic ENKTL-NT. Her condition deteriorated rapidly, with the onset of refractory macrophage activation syndrome leading to death due to multiple organ failure. DISCUSSION: Skin involvement in ENKTL is non-specific and uncommon, which can delay diagnosis. Treatment is based on polychemotherapy comprising L-asparaginase and possibly consolidation therapy with autologous or allogeneic hematopoietic stem cell transplantation. The prognosis of ENKTL-NT is poor due the more aggressive nature of the disease compared with the nasal forms, with frequent visceral involvement and macrophage activation syndrome. Skin involvement seems to be a poor prognostic factor. Although ocular involvement is documented, its association with skin involvement is rare and mainly secondary to nasal forms of ENKTL. This case of an extra-nasal form of ENKTL-NT with systemic involvement illustrates the difficulty of diagnosis and the poor prognosis of this type of lymphoma.

[Characteristics of chronic wounds in substance abuse: A retrospective study of 58 patients]. / Caractéristiques des plaies chroniques chez les toxicomanes : étude rétrospective de 58 patients.

BACKGROUND: Drug addiction causes chronic wounds (CW) responsible for severe complications. Very few studies are available on this topic. The aim of our study was to describe the demographic, clinical and etiological characteristics as well as the course of CW in drug addicts. PATIENTS AND METHODS: This was a retrospective and prospective multicenter study including all drug addicts with CW. RESULTS: We included 58 patients (17 prospectively), 84.5% of whom were male, of median age 43 years, presenting multiple CW as a result of intravenous (78.2%), inhaled (41.1%) and/or snorted (20%) drug abuse. Addiction to opioids (68.4%), cocaine (47.4%) and/or cannabis (40.4%) was ended and/or treated through substitution in 79.3% of patients. CW were fibrinous and necrotic (42.9 to 53.6%), recurrent (54.2%), and in some cases had been present for more than 1 year (61.5%). Intravenous drug addiction was associated with large, fibrinous, ulcers in a setting of venous and lymphatic insufficiency (74%). Only 23% of these wounds involved the upper limbs. Necrotic ulcers associated with clinical arteriopathy were described mainly with inhaled addiction. Abscesses (50%) and erysipelas (29.3%) were the most common cutaneous complications. After 3 months, 50% of CW were improved and 29.2% of patients were lost to follow-up. DISCUSSION: Drug abuse-related CW occurred preferentially in young men with history of intravenous abuse. For the most part, CW were seen on the legs and were associated with venous and lymphatic insufficiency, and the resulting major risk for cutaneous infection increased morbidity and mortality in this population in whom medical follow-up is inherently complicated.

Silver absorption and toxicity evaluation of silver wound dressings in 40 patients with chronic wounds.

BACKGROUND: Silver-containing dressings are considered to be safe even though there have been some reports of complications, including argyria and various organ system dysfunctions. Despite the widespread use of silver dressings, little research has been done regarding the absorption and toxicity of silver. OBJECTIVE: We aimed to study the systemic absorption of silver in patients with chronic inflammatory wounds and to determine associated factors of systemic silver absorption and evaluated its association with silver toxicity. PATIENTS AND METHOD: Prospective, longitudinal, observational, multicentre, open-label pilot study. Patients from the Dermatology Departments of Lorraine (France) with the following inclusion criteria: (i) a chronic wound of more than 6 weeks and (ii) an ulcer needing silver-containing dressing were included. Before and after 28 days of treatment, clinical characteristics of the wound were recorded; hemogram, hepatic and renal functions, albumin sera and serum silver level were measured. RESULTS: Half of the cases displayed raised levels of silver after 1 month of treatment. Predictive factors for systemic silver absorption were wound area, anaemia and malnutrition with anaemia and malnutrition confirmed on multivariate analysis. Wound vascularization may also play a role, as a higher absorption was observed in cases of wound granulation without arterial components. No toxicity was detected. This work has also emphasized the slow elimination of silver from the body. CONCLUSION: Both long-term application and iterative treatments with silver dressings should be discouraged, especially in the elderly, who often suffer from malnutrition and anaemia to avoid potential cumulative toxicity.

[Rare presentations of infantile hemangiomas: 4 cases]. / Présentations rares d'hémangiomes infantiles : 4 cas.

BACKGROUND: Infantile hemangioma (IH) is a common benign vascular tumor in children. In most cases, diagnosis is based entirely on clinical examination. When the diagnosis is uncertain, the first-line complementary examination is Doppler ultrasound. We report 4 cases of atypical infantile hemangiomas with delayed diagnosis and non-contributory imaging. PATIENTS AND METHODS: One child had congenital purple papules and nodules on the back of the foot, the second had inaugural ulceration of the buttocks, and the last two presented telangiectasia, either isolated or on an erythematous macula. In two cases, ultrasound showed no vascular lesions, and in the other two cases, the absence of hyperemia did not allow a diagnosis of IH to be made. For one patient, diagnosis was made on the basis of cutaneous biopsy, and for the other three, on the basis of clinical course. DISCUSSION: We report 4 rare forms of infantile hemangioma resulting in initial diagnostic error. The atypical nature of some IHs may direct the clinician and the radiologist toward other diagnoses that in some cases have no vascular contingent. It is important for the dermatologist to be aware of these rare forms of IH in order to reduce the time to diagnosis and allow early initiation of appropriate management.