Interobserver variability in the classification of childhood maculopapular cutaneous mastocytosis.

BACKGROUND: Maculopapular cutaneous mastocytosis (MPCM) in children is classified in two variants: (i) monomorphic variant, presenting with the small macules or papules typically seen in adult patients; and (ii) polymorphic variant with larger lesions of variable size and shape, typically seen in children. The definition of polymorphic and monomorphic variants is mostly intuitive, and a validation of this classification has not been done. OBJECTIVE: To study interobserver variability in the classification of MPCM in two groups of observers: mastocytosis experts and general dermatologists. MATERIALS AND METHODS: Nineteen cases of childhood MPCM were shown blindly, for classification as monomorphic or polymorphic type, to 10 independent observers (eight dermatologists, one allergist and one haematologist) from Europe and North America with a vast experience in the management of paediatric mastocytosis. Also, the same cases were shown on a screen to 129 general dermatologists attending a meeting; their votes were registered by remote controls. The interobserver variability kappa coefficient (with 95% confidence interval) was calculated to measure the reliability of the correlation. RESULTS: The value of kappa interobserver variability coefficient for the group of 10 experts (95% confidence interval) was 0.39 (0.18-0.63), which is considered as 'fair'. The value of kappa interobserver variability coefficient for the group of 129 general dermatologists (95% confidence interval) was 0.17 (0.06-0.39), which is considered as 'slight'. A complete agreement of all 10 experts was achieved in only four of 19 cases (21.1%) The most voted choice was concordant between the two groups in only 11 of the 19 cases. CONCLUSIONS: We failed to validate the classification system of childhood MPCM in monomorphic and polymorphic types. While the rate of agreement was low for mastocytosis experts, it was nearly the agreement expected by chance in general dermatologists.

Skin manifestations of COVID-19 in children: Part 3.

The current COVID-19 pandemic is caused by the SARS-CoV-2 coronavirus. The initial recognized symptoms were respiratory, sometimes culminating in severe respiratory distress requiring ventilation, and causing death in a percentage of those infected. As time has passed, other symptoms have been recognized. The initial reports of cutaneous manifestations were from Italian dermatologists, probably because Italy was the first European country to be heavily affected by the pandemic. The overall clinical presentation, course and outcome of SARS-CoV-2 infection in children differ from those in adults as do the cutaneous manifestations of childhood. In this review, we summarize the current knowledge on the cutaneous manifestations of COVID-19 in children after thorough and critical review of articles published in the literature and from the personal experience of a large panel of paediatric dermatologists in Europe. In Part 1, we discuss one of the first and most widespread cutaneous manifestations of COVID-19, chilblain-like lesions, and in Part 2 we expanded to other manifestations, including erythema multiforme, urticaria and Kawasaki disease-like inflammatory multisystemic syndrome. In this part of the review, we discuss the histological findings of COVID-19 manifestations, and the testing and management of infected children for both COVID-19 and any other pre-existing conditions.

Skin manifestations of COVID-19 in children: Part 2.

The current COVID-19 pandemic is caused by the SARS-CoV-2 coronavirus. The initial recognized symptoms were respiratory, sometimes culminating in severe respiratory distress requiring ventilation, and causing death in a percentage of those infected. As time has passed, other symptoms have been recognized. The initial reports of cutaneous manifestations were from Italian dermatologists, probably because Italy was the first European country to be heavily affected by the pandemic. The overall clinical presentation, course and outcome of SARS-CoV-2 infection in children differ from those in adults, as do the cutaneous manifestations of childhood. In this review, we summarize the current knowledge on the cutaneous manifestations of COVID-19 in children after thorough and critical review of articles published in the literature and from the personal experience of a large panel of paediatric dermatologists in Europe. In Part 1, we discussed one of the first and most widespread cutaneous manifestations of COVID-19, chilblain-like lesions. In this part of the review, we describe other manifestations, including erythema multiforme, urticaria and Kawasaki disease-like inflammatory multisystemic syndrome. In Part 3, we discuss the histological findings of COVID-19 manifestations, and the testing and management of infected children for both COVID-19 and any other pre-existing conditions.

Skin manifestations of COVID-19 in children: Part 1.

The current COVID-19 pandemic is caused by the SARS-CoV-2 coronavirus. The initial recognized symptoms were respiratory, sometimes culminating in severe respiratory distress requiring ventilation, and causing death in a percentage of those infected. As time has passed, other symptoms have been recognized. The initial reports of cutaneous manifestations were from Italian dermatologists, probably because Italy was the first European country to be heavily affected by the pandemic. The overall clinical presentation, course and outcome of SARS-CoV-2 infection in children differ from those in adults as do the cutaneous manifestations of childhood. In this review, we summarize the current knowledge on the cutaneous manifestations of COVID-19 in children after thorough and critical review of articles published in the literature and from the personal experience of a large panel of paediatric dermatologists in Europe. In Part 1, we discuss one of the first and most widespread cutaneous manifestation of COVID-19, chilblain-like lesions. In Part 2, we review other manifestations, including erythema multiforme, urticaria and Kawasaki disease-like inflammatory multisystemic syndrome, while in Part 3, we discuss the histological findings of COVID-19 manifestations, and the testing and management of infected children, for both COVID-19 and any other pre-existing conditions.

Burden of itch in ichthyosis: a multicentre study in 94 patients.

BACKGROUND: From clinical experience, we know that itch is a major concern for many ichthyosis patients. Nonetheless, no previous studies specifically addressed the issue of itch in ichthyosis. OBJECTIVE: The objective of this study was to specifically address the burden of itch and all its dimensions in ichthyosis patients. METHODS: Ninety-four ichthyosis patients from four different centres were recruited to participate in this cross-sectional, questionnaire-based study. All participants completed the Leuven Itch Scale, a multidimensional self-report instrument that quantifies the frequency, duration, severity, distress, consequences and surface area of itch. RESULTS: Participants included 18 keratinopathic types, 55 autosomal recessive congenital ichthyoses, 11 X-linked recessive ichthyoses (XLRIs), 6 Netherton's ichthyoses, 1 Sjögren-Larsson type, 1 Iocrin ichthyosis and 2 unknown subtypes. Itch occurred in 93% of all patients. In patients with itch, 63% reported that it was often or always present, although most itch episodes were short in duration. Itch, in all its dimensions, was worst in patients with Netherton syndrome. Patients with XLRI had in general a lower itch profile. About half of all ichthyosis patients reported to experience flares during a change in weather, in a hot environment or in stressful situations, whereas a cold environment led to itch in only 26% of patients. The most significant consequences of itching were lesions from scratching, difficulties in falling asleep, bad mood and loss of concentration. CONCLUSIONS: Itch is a major concern in patients with ichthyosis, with significant impact on daily life. Research on future treatments should therefore take itch into consideration and itch should be evaluated in clinical studies. Among the studied subgroups, Netherton patients experienced the most severe consequences.

Topical corticosteroid phobia in atopic dermatitis: International feasibility study of the TOPICOP score.

BACKGROUND: Adherence to topical corticosteroids (TCS) is essential for the effective treatment of atopic dermatitis but can be limited by concerns about their use. This study examined the feasibility of applying the validated TOPICOP score for assessing TCS phobia across different countries. METHODS: This was a prospective multicentre feasibility study conducted in 21 hospitals in 17 countries. Patients >3 months of age with atopic dermatitis or their parents or legal representatives completed a validated translation of the TOPICOP questionnaire in the country's native language. Respondents also completed questionnaires collecting opinions about the feasibility and acceptability of the TOPICOP questionnaire. RESULTS: A total of 1564 participants in 15 countries were included in the analysis. 81% of respondents considered the questions clear or very clear, and 79% reported that it took less than 5 minutes to complete. Each of the individual items in the TOPICOP questionnaire was considered to be not at all difficult to answer by 49% to 74% of participants. The mean global TOPICOP score was 44.7%±20.5. Mean TOPICOP subscores were 37.0±22.8% for knowledge and beliefs, 54.7±27.8% for fears and 50.1±29.1% for behaviours. Global scores and subscores differed between countries, although the subscores did not always vary in parallel, suggesting different levels of TCS phobia and different drivers for each country. CONCLUSIONS: The TOPICOP score can be feasibly applied across countries and may therefore be useful for obtaining qualitative and quantitative data from international studies and for adapting patient education and treatment.

Burden of itch in epidermolysis bullosa.

BACKGROUND: Itch is an unpleasant feeling that leads to scratching. It is a common, but understudied, problem in patients with epidermolysis bullosa (EB). OBJECTIVES: We measured the prevalence and characteristics of itch in the three major forms of EB: generalized EB simplex (EBS), junctional EB (JEB) and dystrophic EB (DEB). METHODS: Forty patients with EB were recruited from two tertiary care centres and one patient organization. The sample included 19 patients with EBS, seven with JEB and 14 with DEB. Patients completed the Leuven Itch Scale (LIS), a multidimensional self-report instrument that quantifies the frequency, duration, severity, distress, consequences and surface area of itch. This instrument has good clinimetric properties. RESULTS: Itch occurred in 85% of the patients, with substantial differences across the subtypes (EBS 74%, JEB 100%, DEB 93%). Itch, in all its dimensions, was most pronounced in patients with JEB and DEB, and less prominent in patients with EBS. The scores were significantly different for itch frequency, severity, distress and surface area, and the overall itch scores were comparable with those of atopic dermatitis. Itch mainly occurred in a hot environment (65%) and when sweating (62%). The most prevalent consequences were difficulty in falling asleep (88%) and lesions from scratching (85%). Differences between the three major subtypes were also observed in terms of circumstances, consequences and sensory characteristics. CONCLUSIONS: As expected, itch is common among patients with EB. All aspects of itch measured by the LIS were more severe in JEB and DEB than in EBS.

[Lack of compliance to topical corticosteroid therapy: possible cause and solutions]. / Manque d'observance thérapeutique a la corticothérapie topique: causes et solutions possibles.

Adherence to therapy is low for topical therapy used in dermatological disorders. particularly in chronic diseases like atopic dermatitis and psoriasis. One of the reasons is that patients do not trust their therapy and fear side effects, particularly with topical corticosteroids. In order to make patients more confident, it is it important to provide correct and detailed information about the prescribed products and the amount to apply, to involve them in the implementation of therapy and to harmonize the information given by various care providers (physicians, nurses, pharmacists). The message needs to be clear and consistent between caregivers and not lead to unjustified worries. Poor adherence may result in the use of stronger preparations or switch to systemic treatment, which eventually will result in more severe side effects.

Atopic dermatitis: triggering factors.

Atopic dermatitis is a hereditary disorder, frequently associated with allergic rhinitis and bronchial asthma. The disease may be influenced by many triggering factors such as irritants, aeroallergens, food, microbial organisms, sex hormones, stress factors, sweating, and climatologic factors. Moreover, it is important to be aware of contact allergy as a complicating factor. This review deals with recent clinical, experimental, and some therapeutic data on these triggering factors.

Acanthosis nigricans caused by nicotinic acid: case report and review of the literature.

Drug-induced acanthosis nigricans has been reported in the literature. We present a patient with familial combined hyperlipidemia who developed nicotinic-acid-induced acanthosis nigricans. The literature on the cutaneous side effects of nicotinic acid as well as on the medications that can cause acanthosis nigricans is reviewed. Some hypotheses on the pathogenesis of nicotinic-acid-induced acanthosis are presented.

alpha-Amylase, a flour additive: an important cause of protein contact dermatitis in bakers.

BACKGROUND: alpha-Amylase, an enzyme commonly used in flour additives, has been reported to be an important cause of rhinitis and asthma in bakers. OBJECTIVE: Our purpose was to determine whether this enzyme could also cause dermatitis. We tested it routinely in bakers with hand eczema. METHODS: Patch tests were administered with the International Contact Dermatitis Research Group standard series and a bakery series and scratch-chamber or prick tests were performed with the bakers' own material and with alpha-amylase powder. RESULTS: Of 32 bakers tested, seven had an immediate wheal-and-flare reaction and two also had a delayed eczematous reaction. High dilutions of the alpha-amylase powder still gave strong reactions. CONCLUSION: alpha-Amylase is an important cause of skin reactions in bakers and should be tested routinely if a contact allergy is suspected.

[Comment on the contribution by L. Weber: Yersinia exanthema]. / Stellungnahme zur Arbeit von L. Weber: Das Yersinien-Exanthem.

Lutz Weber has described Yersinia exanthema, a distinctive dermatological disorder characterized by three or all of the following signs: 1. A typical erythema-multiforme-like eruption, particularly on the neck, shoulders, and arms, 2. Erythema nodosum, 3. Conjunctivitis, particularly on the nasal side of the conjunctiva, 4. Arthralgia. The case presented here concerns a patient who satisfied all of the criteria for Yersinia exanthema and in whom no infection could be demonstrated.

Contact allergy to isothiazolinone derivatives: unusual clinical presentations.

Contact allergic reactions to the mixture of 5-chloro-2-methyl-4-isothiazoline-3-one and 2-methyl-4-isothiazoline-3-one are most frequently associated with intolerance to cosmetics. The present article points out that such reactions, particularly on the face, can have unusual clinical presentations that are very similar to seborrheic eczema, lupus erythematosus, lymphocytic infiltrate or photodermatitis. Atopic dermatitis is also often erroneously suspected.