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Reports written by Dr. Déborah Salik (Dermatologist, Belgium), Dr. Joséfina Marco Bonnet (Dermatologist, France), Dr. Laura Bouchard (Dermatologist, Finland), Dr. Joël Claveau (Dermatologist, Quebec) and Dr. Ibrahim Fayez (Dermatologist, Canada)
By
Dr. Déborah Salik In collaboration with 4 other professionals
Related topics
Speakers: Prof. Carle Paul, Dr. Myrto Trakatelli and Dr. Lise Boussemart
Reports written by Dr. Déborah Salik
To start, the participants introduced themselves and said what they expected to get out of the workshop.
Next, they brainstormed to establish the core values of the workshop: trust, caring, listening, kindness, indulgence, patience, firmness, respect, experience, impartiality, confidentiality, sharing emotions, honesty, self-criticism, tolerance, reflection, spontaneity, efficiency, non-judgement.
“Leadership is the art of persuading people to work toward a common goal” - D. Goleman
The concept of social intelligence was first brought to light by E. Thorndike.
In 1980: H. Gardner introduced the concept of multiple intelligences.
In 1990: P. Salovey and J. D. Mayer coined the term “emotional intelligence”.
In 2000: R. Boyatzis and D. Goleman developed a model of emotional intelligence, broken down into domains and competencies, and evaluated and validated the tool.
The vast majority of the skills developed in higher education are cognitive skills. They provide a core set of technical skills leading to a diploma. Future differentiation will be based on emotional skills, i.e.
The dimensions of emotional balance according to R. Davidson are as follows:
There are 12 competencies of emotional intelligence:
The key domains in social and emotional intelligence are as follows:
There are various styles of leadership: coercive, authoritative/visionary, democratic/inclusive, and coaching/mentoring.
The main objectives in leadership are trust, authenticity, empathy, and logic. Trust is central to building relationships in leadership.
The concept of leadership is influenced by personal impact. This is the positive or negative individual impact we have as a leader in a relationship.
There is no impact on age, gender, nationality, culture, biography or reputation, but this is part of personal impact.
However, certain aspects can be modulated: physical presence, clothing, appearance, listening skills, competence, language and choice of words, politeness, and courtesy.
There are three levels of listening
Similarly, there are various types of listeners. We can adopt an attentive, intermittent, distracted, weak, elective, opportunistic, confirmation bias, deficit-based, contradictory, or convergent approach.
In listening and understanding, some cues can be important, such as eye contact, facial expressions, nodding, and body language. The listener should avoid interrupting and external distractions (text messages, telephone calls) should be eliminated. No judgement, no interpretation.
It is necessary to create a transition cycle and establish a connection. We attach to a secure base. We establish connections with people, values, and objectives. When change occurs, we are separated from the things and people to which/whom we are connected. We experience emotions in response to change that vary in intensity.
Unresolved grief/losses can prevent us from fully connecting with other people.
A secure base leader may be a person, a connection, or a goal – something that creates a feeling of protection. They are a source of energy that will inspire us and enable us to take risks and achieve fulfilment. We can take the example of a parent and their child.
The components of our secure base are essential for learning and making sense of things; they influence the way we think.
A secure base leader offers help. They are a good listener who deciphers verbal and non-verbal cues and is attentive to needs. Rather than defending a position, they ask questions to learn more. They do not think for others. “They have unconditional positive regard toward the other person” - C. Roger. They see potential, even if the other person does not. They are reliable and available to the other person and will encourage them if they have any problems.
Empathy is the intuitive ability to put ourselves in another person’s shoes and sense what they are feeling. It is a powerful tool for emotional intelligence.
There are three types of empathy:
Empathy helps us understand other people and ourselves. It is essential in human relations and in doctor-patient and leader-team relationships.
It is a skill that can be developed through mirror neurons.
Here are a few techniques for cultivating empathy during a consultation:
There are no right or wrong answers here.
It is important to create space to encourage exploration and ask open-ended and hypothetical questions, to redirect the attention of the person asking the question. This helps clarify the unknown and lowers the stakes when it comes to deciding whether to adopt a particular course of action; it also invites us to consider different possibilities.
There are various types of questions to ask:
Conflict can be defined as a difference between two or more people, causing tension, an emotional response or disagreement when trust is broken or absent.
Conflict is caused by the breakdown of a relationship or the inability to deal with loss or grief.
Who am I when faced with a conflict?
Analyse the conflict, and identify the
What is the other person’s goal? Where is the tension?
What are your areas of common interest?
To negotiate with “difficult” people, we can try using the “noble storytelling” technique, considering the:
There are also eight principles of conflict management:
Speakers : Dr. C. Devin, Prof. Florent Grange, Dr. Aude Nassif, Dr. Laura Fertitta and Dr. Arnaud Porquet
Report written by Dr. Déborah Salik
Mastocytosis is a disorder characterised by mast cell infiltration in organs. The WHO classification distinguishes between cutaneous mastocytosis and systemic mastocytosis (indolent, aggressive, with clonal haematological disease).
The pathophysiology is generally known as being linked to a mutation in the KITD816V gene, but this alone does not explain the different phenotypes of the disease. Other genes and signalling pathways are probably involved
This study focused on a patient with a form of congenital aggressive systemic mastocytosis, which accounts for less than 0.05% of mastocytosis cases and could be genetic in origin. He had a KIT mutation, multi-organ involvement (skin, bone marrow, liver, spleen), and recurrent anaphylaxis
An analysis of his exome found a homozygous, loss-of-function SLC26A2 variant (R279X (c.C835T, pArg279Trp). This variant is responsible for a sulphation defect in solid cancers, which probably plays a role in cancer plasticity. Inhibition of sulphation increases KIT proliferation and phosphorylation.
Sulphate reduces KIT proliferation and phosphorylation.
A second transcriptomic study in 33 patients with sporadic systemic mastocytosis showed a decrease in the PAPSS2 transcript (tumour suppressor gene).
These two genes regulate cell proliferation and tyrosine phosphorylation and could be new therapeutic targets in mastocytosis.
XP is a particularly rare condition in Nepal. All the patients affected exclusively have the XPC pR415X mutation in its homozygous state, suggesting a founder effect of this mutation. This variant is characterised by the occurrence of extremely early carcinomas.
The aim of this study was to assess the preventive habits of XP patients in Nepal:
There is therefore a lack of knowledge as well as inadequate access to healthcare and sun protection. The obstacles are related to poverty, lack of health insurance, early school leaving, social exclusion, isolation, and distance from medical clinics. There is therefore a need to inform and educate the population (parents, families, youth workers) and put in place social and community assistance.
This study helped identify needs in order to improve the provision of care.
The genetics of Verneuil’s disease still remain unclear.
We reported an association between HS and gene variants involved in immune deficiency and/or immune response.
Regarding genetic factors, the following have been found:
A new hypothesis has been put forward: HS may be associated with immune deficiency genes.
Neurofibromatosis type 1 (NF1) is an autosomal dominant genodermatosis linked to mutations in the NF1 gene (tumour suppressor gene).
There is a constant presence of tumours such as neurofibromas (NFs), which are
Patients develop malignant peripheral nerve sheath tumours (MPNSTs), which are the main cause of death. The formation of these MPNSTs appears to be based on the conversion of NFp to dysplastic NFs.
The number of NFsc is independent of mortality, but if the number of NFsc is greater than 10, then there is a risk associated with the presence of NFi.
A risk phenotype can therefore be described in the presence of one NFi or more than 10 NFsc. The aim of this study was to investigate the mortality of NF1 patients as a function of these phenotypes.
Case-control studies in patients with at least two NFsc:
Results:
The presence of NFsc > 2 was associated with a significant increase in mortality (five MPNSTs among the patients who died).
The prevalence of vascular dysplasias (VDs) in NF1 ranges from 0.4 to 6.4%.
The most common are renal artery stenosis and arterial aneurysm, with frequent arterial disease. The complications can include stenosis, aortic narrowing, Moyamoya disease or a life-threatening aneurysm/pseudoaneurysm.
The frequency of these abnormalities is unknown and there are no recommendations for screening.
The study presented was conducted on 123 patients with 210 vascular dysplasias.
Study results:
In conclusion:
VD is common (18.5%) and is often asymptomatic in NF1 patients. In the event of optic pathway glioma, systematic screening for cerebral VDs is necessary. If a VD is present, screening for VDs in other regions should be performed.
Speakers: Dr. M. Saint-Jean, Dr. Lucie Peuvrel and Dr. C. Abadie
Report written by Dr. Déborah Salik
The basic working tool is the patient’s family pedigree, which is created during the consultation. Based on this consultation, we can determine whether there is a suspected genetic predisposition:
The oncogenetic consultation should be carried out with the patient’s agreement and informed consent.
The genetic test involves DNA sequencing from blood and a buccal smear, followed by a laboratory analysis of the DNA sequencing results, after which the patient is called in for a consultation.
The consultation takes place in parallel with a consultation with a psychologist.
If a genetic predisposition is identified:
Classification of variants (ACMG classification):
If a genetic predisposition is identified, there are:
It should be noted that the family has an obligation to inform other family members.
Obstacles in oncogenetic consultations include:
There are:
The genetic panel for melanoma is as follows: CDKN2A - CDK4 - BAP1 - MITF - MC1R - ACD - POT1 - TERT and TERF2IP
(+ initial digital video-dermoscopy at M0, M3, M12 and then half-yearly, and annual whole-body imaging)
Rare variant
Leading tumour spectrum
Monitoring from the age of 18:
Monitoring:
Clinical signs:
Mucocutaneous lesions (>95%)
Thyroid involvement >70%
Digestive involvement
Mammary involvement (>65%)
Urogenital involvement
Neurological involvement (40%)
Incidence: 8-9/100,000
Genes coding for TSC1 and TSC2, autosomal dominant transmission
Reduced life expectancy
Clinical manifestations:
Good genotype/phenotype correlation but possibility of a mosaic form of tuberous sclerosis complex.
Monitoring should include an annual dermatological examination.
As regards drug treatments, prescription of:
1/3,000 births
Complete penetrance at eight years of age; children have almost all (97%) the diagnostic criteria.
Highly variable phenotype.
Mutation in a tumour suppressor gene: NF1 acts in the MAP-kinase pathway, enabling cells to survive and proliferate.
Neurofibromin negatively regulates the RAS pathway by converting active RAS-GTP into inactive RAS-GDP.
Kinetics of the lesions
Before one year: characteristic bone lesions and café au lait spots
Three years: Lisch nodules
Four to six years: optic pathway glioma or lentigines
Investigation of complications during:
The presence of vesicles or bullae is rare in Kawasaki disease and should lead to the consideration of other diagnoses.
This means we should watch out for the polymorphism of mucocutaneous eruptions in Kawasaki disease.
Toxic shock syndrome
Bioderma Congress Reports EADV 2023
Bioderma Congress Reports WCD 2023
