Childhood epidermal necrolysis and erythema multiforme major: a multicentre French cohort study of 62 patients.

INTRODUCTION: The distinction between epidermal necrolysis [EN; including Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN) and overlap syndrome] and erythema multiforme major (EMM) in children is confusing. We aimed to better describe and compare these entities. MATERIALS AND METHODS: This French retrospective multicentre study included children ≤18 years old referred for EN or EMM between 1 January 2008 and 1 March 2019. According to pictures, children were reclassified into TEN/overlap, SJS or EMM/unclassified (SJS/EMM) groups and compared for epidemiological and clinical data, triggers, histology and follow-up. RESULTS: We included 62 children [43 boys, median age 10 years (range 3-18)]: 16 with TEN/overlap, 11 SJS and 35 EMM. The main aetiologies were drugs in EN and infections (especially Mycoplasma pneumoniae) in EMM (P < 0.001), but 35% of cases remained idiopathic (TEN/overlap, 47%; SJS, 24%; EMM, 34%). The typical target lesions predominated in EMM (P < 0.001), the trunk was more often affected in EN (P < 0.001), and the body surface area involved was more extensive in EN (P < 0.001). Mucosal involvement did not differ between the groups. Two patients with idiopathic TEN died. Histology of EMM and EN showed similar features. The recurrence rate was 42% with EMM, 7% with TEN/overlap and 0 with SJS (P < 0.001). Sequelae occurred in 75% of EN but involved 55% of EMM. CONCLUSION: Clinical features of EN and EMM appeared well demarcated, with few overlapping cases. Idiopathic forms were frequent, especially for EN, meaning that a wide and thorough infectious screening, repeated if needed, is indicated for all paediatric cases of EN/EMM without any trigger drug. We propose a comprehensive panel of investigations which could be a standard work-up in such situation. Sequelae affected both EN and EMM.

Interest of corrective makeup in the management of patients in dermatology.

BACKGROUND: Disfiguring dermatoses may have a significant impact on patients' quality of life, namely on their relationship with others, self image, and self esteem. Some previous studies have suggested that corrective foundation can improve the quality of life (QOL) of patients with facial dermatoses; in particular, in patients with acne vulgaris or pigmentary disorders. OBJECTIVE: The aim of this prospective study was to evaluate the impact of the skin conditions of patients with various skin diseases affecting their face (scars, acne, rosacea, melasma, vitiligo, hypo or hyperpigmentation, lentigines, etc) on their QOL and the improvement afforded by the use of corrective makeup for 1 month after being instructed on how to use it by a medical cosmetician during an initial medical consultation. METHODS: One hundred and twenty-nine patients with various skin diseases affecting the patients' face were investigated. The patients were instructed by a cosmetician on how to use corrective makeup (complexion, eyes, and lips) and applied it for 1 month. The safety of the makeup application was evaluated and the QOL was assessed via a questionnaire (DLQI) and using a 10-cm visual analog scale (VAS) completed before the first application and at the final visit. The amelioration of their appearance was documented by standardized photography. RESULTS: No side effects occurred during the course of the study. A comparison of the standardized photographs taken at each visit showed the patients' significant improvement in appearance due to the application of corrective makeup. The mean DLQI score dropped significantly from 9.90 ± 0.73 to 3.49 ± 0.40 (P < 0.0001). CONCLUSION: Our results suggest that dermatologists should encourage patients with disfiguring dermatoses to utilize appropriate and safe makeup to improve their appearance and their QOL. Corrective makeup can also complement the treatment of face dermatological diseases in order to improve patient's adherence.

[Overview of five years of occupational dermatology: The role of atopy]. / Consultation spécialisée de dermatologie professionnelle : bilan de cinq ans d'activité. Place de l'atopie.

BACKGROUND: The management of occupational dermatitis is often complex and relies on skills in both dermatology and occupational medicine. A bidisciplinary occupational dermatology consultation was set up in 2002 at Brest CHU. The aim of this study is to provide an assessment of 5 years of activity and to discuss the role of atopy. PATIENTS AND METHODS: From October 2002 to October 2007, 245 patients attended this consultation. 145 files concerning proven occupational dermatosis were studied (age, sex, occupation, dermatosis, allergens and history of atopy). One hundred and five patients completed a telephone questionnaire concerning their feelings about the consultation, the course of their dermatosis and the medicosocial outcome. RESULTS: The main forms of dermatosis were contact eczema (41%), irritant dermatitis (26%), exacerbation of atopic dermatitis (10%), associated eczema and irritant dermatitis (9%), and other (14%). DISCUSSION: The occupations of subjects (healthcare, building and civil engineering work, food and catering, agriculture, mechanics, hairdressing, cleaning, printing) and the allergens involved were similar to those described in the literature. The prognosis was poor (48% cure rate). Differential diagnosis between allergic eczema and irritant dermatitis was sometimes difficult and was based on clinical features and allergology tests. A history of atopy was found in 32% of patients and was particularly frequent among patients presenting allergic eczema associated with irritant dermatitis: this may be explained by the physiopathological mechanisms of atopic dermatitis (impairment of barrier function causing contact sensitization to potential allergens). CONCLUSION: Such a bidiscipinary occupational dermatology consultation has the advantage of optimizing management of these patients. Wider notification of occupational dermatosis would increase our knowledge of the causes of this condition and enable better prevention.

Frequency of aquagenic palmoplantar keratoderma in cystic fibrosis: a new sign of cystic fibrosis?

BACKGROUND: Aquagenic palmoplantar keratoderma (APPK), also known as aquagenic wrinkling of the palms, is characterized by oedema of palms and/or soles, whitish papules, hyperwrinkling and sometimes pruritus or pain after water immersion. Its frequency in the general population is unknown. About 40 cases have been reported to date, including some among patients with cystic fibrosis (CF) or CF heterozygotes. OBJECTIVES: To determine the frequency of APPK among patients with CF. METHODS: Twenty-seven patients from the Centre of Competence on Cystic Fibrosis of Roscoff were examined by a dermatologist after immersion of the palms in water for 2-3 min. RESULTS: The frequency of APPK was 41% (11 of 27 patients). Some patients had not previously noticed the lesions. The frequency was higher among inpatients than outpatients. We suspect that occlusion (caused by the gloves worn by inpatients) can explain this difference. The number of patients included in this study is not sufficient to draw any conclusions concerning the type of CF mutation and its impact on the frequency of APPK. CONCLUSIONS: APPK is frequent among patients with CF and, thus, should be considered a sign of CF. APPK is underdiagnosed because physicians usually do not look for it. CF screening should be considered for any patient presenting with these symptoms, followed by genetic counselling if necessary.