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Reports written by Dr. Nives Pustisek (Pediatric dermatologist, Croatia), Prof. Ivelina Yordanova (Dermatologist, Bulgaria) and Dr. Rémi Maghia (Dermatologist, France)
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Dear colleagues,
It was my pleasure and honor to participate in the first ESPD meeting in person since the pandemic. We have all accepted that different video platforms are serving the purpose, however contact in person and live discussions are still the top choice when talking about efficient transfer of knowledge and deepening the relationship among colleagues professionals. Having said that, let me compliment an excellent scientific program and opportunity to socialize with colleagues during the ESPD meeting in Munich this year. As you are aware, the program went on in two parallel sessions. Please find below several reviews of very interesting lectures.
Speakers: Prof. Antonio Torrelo, Prof. Milos Nikolic and Prof. Henning Hamm
Report written by Dr. Nives Pustisek
Prof. Antonio Torrelo (Spain)
The lecture began with the classification of autoinflammatory diseases (Dilan Dissanyake, SPD meeting 2021, Toronto): inflammasomopathy, interferonopathy, NF-kBopathy. Each group shared some clinical characteristics, variable phenotypes and treatment options. Inflammasomopathy are a heterogeneous group of genetic diseases, with variable cytokine profiles (IL-1ß, IL-18, IL-36) and they are called IL-1 driven autoinflammatory diseases. Clinical characteristics of inflamasomopathy include fevers, organ involvement (abdominal pain, non-vasculitis rashes, uveitis, arthritis), elevated WBC/neutrophils, highly elevated inflammatory markers. This group is associated with variable phenotypes (urticarial, edematous, pustular, neutrophilic, psoriasiform and others) and variable systemic signs. Treatment includes IL-1 blockade (anakinra, rilonacept, canakinumab), IL-18 blockade (tadekinig-alfa-IL-18BP), IL-36 (spesolimab). Vey interesting case reports have been presented, such as Criopyrin associated periodic syndromes (CAPS; NLRP3 pathogenic change identified) and Deficiency of IL-36 receptor antagonist (DITRA). Clinical characteristics of interferonopathy include fevers, organ involvement (vasculitic rashes, interstitial lung disease, intracranial calcifications), inflammatory markers may not be as elevated, autoantibodies may be present. They are treated by JAK inhibitors. In this group of patients, as well, interesting cases have been presented and a list of major monogenic interferonopathy and skin features. Clinical characteristics of NF-kBopathy include fevers, highly variable organ involvement (oral/GI/GU ulceration, granulomas).
In conclusion, we can suspect an autoinflammatory disease when we are presented with a non- infectious periodic fever and skin lesions; skin lesions triggered by common triggers; early onset connective tissue disease; recurrent panniculitis in a young child; cutaneous vasculitis and systemic signs; unexpected skin lesions and systemic signs; inflammatory skin disease with minimal lab anomalies; severe, intractable disorder of keratinization; skin lesions of infancy with predominant neutrophils; unusual skin lesions and immunodeficiency and cytopenias. Additionally, another sign that the disease is of autoinflammatory nature is when we’re not sure what it is. Around 50 well- characterized autoinflammatory disease exist. But still, only around 50% of patients with autoinflammatory diseases have a gene.
Prof. Milos Nikolic (Serbia)
Juvenile dermatomyositis (JDM) is a rare, serious systemic autoimmune disease, immune occlusive small-vessel vasculopathy. It’s the most common juvenile idiopathic inflammatory myopathy that affects skin, skeletal muscle, joints, gastrointestinal tract, heart and lungs. Some patients (1-5%) have amyopathic JDM (of these 25% developed overt JDM). 1/3 patients have acute, monocyclic course (up to 2 years); ¼ have polycyclic disease. More than 50% have chronic, continuous disease despite treatment. Even after 16 years of evolution, more then 50% of JDM patients still have an active disease (capillary nailfold changes, skin rash and others). Before the steroid era, 1/3 patients died, 1/3 had significant disability and 1/3 completely recovered. Today the mortality rate is more then 2%. JDM is generally not paraneoplastic. Very rare paraneoplastic cases have been presented in the literature. The incidence is 1-4 / 1 000 000 children/year, girls:boys 3:1. Etiopathogenesis is complex, immunological dysfunction (genetic background) and environmental stimuli / triggers (infection, drugs, UV light etc.). Some antibodies have the same association as in adults: anti-PM-Scl (overlap with scleroderma), anti-Mi-2 (classic skin rash, milder muscle involvement, lower risk of interstitial pneumonia, respond well to therapy), ant-Jo-1 (interstitial lung disease). Some antibodies are associated with a mild form of disease like anti-MDAS and anti-SAE. Anti-Ro (SSA) are associated with poor prognosis. The new Classification criteria 2017 for adult and juvenile inflammatory myopathies has been presented. The second part of the presentation included patients with JDM from the Department of Dermatology, Belgrade, 1990-2021, clinical presentation, disease course and the complexity of treatment options.
Prof. Henning Hamm (Germany)
Pediatric psoriasis is a systemic inflammatory immune-mediated disease. 30% of patients have disease onset before the age of 20 years. Prevalence in the European children and adolescents 0.5- 1%. Pediatric psoriasis shows a strong genetic predisposition, most important PSORS1, HLA-Cw6. Pathogenesis is very complex, stimulation of keratinocytes by proinflammatory cytokines (T cells: IL- 17A, IL 6, IL8, TNFα; dendritic cells: IL-1ß, IL-12, IL-23, THFα). Pediatric psoriasis is associated with a number of comorbidities (metabolic, cardiovascular, articular, gastrointestinal and psychological, most important obesity). It has a strong influence of patient quality of life and also parents and family quality of life. Indication for systemic treatment, moderate to severe psoriasis defined by Psoriasis Area and Severity Index (PASI) ≥10, Body Surface Area (BSA) ≥10, Children’s Dermatology Life Quality Index (CDLQI) or Dermatology Life Quality Index (DLQI) ≥16 years ≥10; predominant involvement of sensitive sites (scalp, face, palms, soles, nails, genital and intertriginous areas) not sufficiently considered in PASI and BSA. Five biologic agents have been approved for pediatric psoriasis (etanercept, adalimumab, ustekinumab, ixekinumab, scukunumab). The German guidelines update 2021 and diagnostic criteria for pediatric psoriasis have also been presented. The clinical presentation and treatment for Generalized pustular psoriasis annular type has also been presented. “Psoriasis dermatitis” is an overlap condition of psoriasis and atopic dermatitis. Psoriasiform dermatitis during dupilumab treatment and paradoxal psoriasis (psoriasifom lesions induced by biologics, mainly TNF-alfa inhibitors) have been presented.
Speakers: Prof. May El Hachem, Prof. Stephanie Christen-Zäch and Prof. Antonella Muraro
Report written by Dr. Nives Pustisek
Prof. May El Hachem (Italy)
Photosensitivity is an abnormal response to sun exposure or to an artificial light source, with variable skin eruption manifestations and pruritus. The presentation included a classification of photosensitivity: primary photosensitivity (Polymorphous light eruption, Actinic pruritus, Juvenile spring eruption, Solar urticaria, Hydroa vacciniforme), due to exogenous agents (Drug induced, Phytophotodermatitis), connective tissue diseases (Neonatal lupus erythematosus, Systemic lupus erythematosus, Juvenile dermatomyositis), photoaggravated dermatoses (Herpes simples, Psoriasis, Atopic dermatitis, lichen, Darier’s disease), different metabolic disorders (Erythropoetic protoporphyria, Congenital erythropoetic porphyria and others) and genodermatoses (Trichothiodystropy, Xeroderma pigmentosum, Rothmund-Thomson syndrome, Kindler EB and others). Through interesting case reports different photosensitivity disorders have been presented with differential diagnoses, diagnostic and therapy. The diagnostic work up includes clinical and family history (date of onset of the lesions, relationship with sun exposure, evolution, drug assumption or use of topical product, associated disorders, family history (genodermatoses, connective diseases, etc.), clinical exam, laboratory tests (depending on the clinical diagnostic hypothesis), phototesting (in selected cases), skin biopsy and other investigations.
In conclusion, photosensitivity is present in several disorders, it may exacerbate multiple common diseases. Accurate clinical history and exam should be performed. Early diagnosis is required to avoid complications and to guarantee an appropriate therapy, differential diagnosis should be always considered. Diagnostic management depends on the clinical diagnostic hypothesis. Multidisciplinary management is frequently necessary.
Prof. Stephanie Christen-Zäch (Switzerland)
Etiology of urticaria and angioedema: infections, allergic (food, drugs, contact allergens, aeroallergens), pseudo-allergic (aspirin, NSAIs, food additives, local anesthesia, radiocontrast agents), physical (mechanic, light, cold/heat), toxic (insects, plants, jellyfish, chemical substances), cholinergic (when exercising), endogenic (auto immune, auto-inflammatory, hematologic) and idiopathic. If one is presented with an angioedema without urticaria, this could point to C1 esterase inhibitor deficiency, angiotensine-converting enzyme inhibitors, hereditary angioedema (bradikinin mediated). Clinical presentation of urticaria can be in the classical form, figurated urticaria, dermographismus, urticarial papules (cholinergic urticaria), giant urticaria (in babies, appearance like a bruise, frequent location on hands and feet). Urticaria vasculitis, Serum sickness reactions, Acute hemorrhagic edema of infancy, Maculopapular, cutaneous mastocytosis, Auto-inflammatory syndromes, Nonmast cell mediator-mediated angioedema can be manifested with wheals or angioedema, but not considered to be subtypes of urticaria as they have different pathophysiologic mechanisms. Urticaria classification: acute urticaria (less than 6 weeks), chronic urticaria (more than 6 weeks), spontaneous urticaria (no specific eliciting factor involved) and inducible urticaria (specific eliciting factor involved). Chronic urticaria subtypes can be chronic spontaneous urticaria (CSU) and chronic inducible urticaria (physical or other). Duration of CSU is estimated to be 1-5 years in most cases. In diagnostic work up of urticaria one should not do intensive and costly general screening program, not all possible causative factors need to be investigated in all patients. The most important thing is patient’s history, physical examination and in some patients when is indicated further appropriate diagnostic tests (look in diagnostic algorithm for chronic urticaria (Allergy 2018). In the final part of the lecture, the treatment algorithm for urticaria according EAACI/GA2LEN/EDF/WAO guidelines (Allergy, 2018) and strong influence of chronic urticaria on quality of life have been presented.
Prof. Antonella Muraro (Italy)
The lecture exhibited a connection between food allergy and atopic dermatitis (an in-parallel journey?), pathophysiology and the link between food allergy and atopic dermatitis: epicutaneous sensitization, and diagnostic work-up of food allergy in atopic dermatitis and prevention strategies. Food allergies and atopic dermatitis appear to be closely associated, both represent a public health burden with a huge psychosocial impact on children and their families. In children below 6 years of age with mild atopic dermatitis, a diagnostic work up for food allergy may be warranted if the history is positive for immediate reaction to food. In moderate atopic dermatitis if there is a poor response to an adequate local treatment or positive history for immediate reaction to food. Introduction of egg and peanut early between 4 and 6 months of age is suggested to prevent food allergy especially in high risk children with mild atopic dermatitis and in countries with high prevalence of peanut allergy. Optimizing the care of the skin is paramount to achieve a good control of food allergy and possibly to prevent further sensitization. Recent findings on the endotypes and ILRA variant add further support to the dual hypothesis of sensitization vs tolerance and pave the way for the use of biologicals active on the TH2 pathways of the allergic inflammation for reducing food allergy and atopic dermatitis. In conclusion, the ultimate goal is to change the natural history of food allergy and atopic dermatitis preventing the progress of the allergic disease i.e. the “Atopic March”.
Speakers: Prof. Dirk van Gysel, Prof. Klara Martinaskova and Prof. Regina Fölster-Holst
Report written by Dr. Nives Pustisek
Prof. Dirk van Gysel (Belgium)
The definition and classification of exanthems has been presented in the introduction. Exanthems are acute mucocutaneous dermatoses with erythema (vasodilatation), urticaria (oedema, extravasation), papules (cellular infiltrate) and purpura (vessel wall damage). Exanthems can be viral (measelses, rubella, erythema infectiosum, exanthema subitem, varicella, enterovirus infection, adenovirus infection, EBV infection, HFM disease), bacterial (impetigo, SSSS, scarlet fever, meningococcal sepsis, Rocky Mountain spotted fever) and of various types (Gianotti- Crosti syndrome, Kawasaki syndrome, APEC, EEM – Stevens-Johnson syndrome). During the lecture, very good photos and case reports of old exanthems (classic presentation and atypical/new presentation) and new exanthems (due to novel viruses and due to population and vector movement) have been presented. For example, measles can be presented as classical measles, atypical measles, attenuated measles 6 days after vaccination and modified measles after 2 previous doses of MMR vaccine. Varicella is a very common viral exanthems caused by varicella zoster virus (HHV-3) and can be complicated as hemorrhagic varicella and bacterial superinfection. Herpes zoster is reactivation of varicella zoster virus and characterized with preeruptive, acute eruptive and chronic phase. Hutchinson’s sign in herpes zoster infection means skin lesions at the tip, the side and the root of the nose, representing the dermatomes of the external nasal and infratrochlear branches of the nasociliar nerve Hutchinson’s sign is associated with increased likelihood of ocular complications associated with infection. Ramsay Hunt syndrome (herpes zoster oticus) is reactivation of Varicella zoster virus involving the facial and auditory nerves. Exanthems with infectious mononucleosis can be in 3 patterns: maculopapular (in young child), morbililiform eruptions (associated with antibiotics, ampi or amoxi), and Gianotti-Crosti syndrome. The clinical presentations of Asymetric periflexural exanthema of childhood, associated skin manifestations with Epstein-Barr virus, Hand foot and mouth disease, Papular purpuric gloves and socks syndrome have been presented. Finally, the skin manifestation of COVID-19 (acute COVID 19, COVID-19 vaccine induced skin lesions) and travel-related diseases (imported dermatoses) have been presented.
In conclusion, different agents (viruses, drugs etc.) can cause a similar eruption and one single agent (virus, drugs etc.) can cause different eruptions. The clinical picture is often clear-cut. In some cases, a systematic approach is necessary to make the right diagnosis. Diagnostic approach in viral exanthems include history (age, season, drug intake, outbreaks, prodromal phase, fever, other complaints or symptoms, travel dates and destination), physical examination then laboratory and technical investigations.
Prof. Klara Martinaskova (Slovakia)
Exanthems in children are extremely common and its manifestations range from mild to severe diseases. An exanthem is defined as any eruptive skin rash that may be associated with lesions of the mucous membranes (enanthem), fever or others symptoms. Exanthem may be a manifestation of an infectious disease or an adverse reaction to drugs. Risk of severity of exanthema can be associated with primary or secondary immunodeficiency, genetic disorders, malignancy, immunopresive treatment, co-infection, post COVID syndrome. Co-infection happens when in some situation at the same time period we detect not only one pathogen. Some authors distinguish concomitant/co-infections and superinfections. Co-infection is occurring concurrently with the initial infection, while superinfections are those infections that follow on a previous infection. The cases of co-infections Hand foot and mouth disease with adenovirus, co- infections Parvovirus B19 and Mycopasma pneumonia, co-infection Enterovirosis B6, Mycoplasma pneumonie and Parvovirus B19 in imumunocomprimised 10-year old girl and others have been presented, as well as case reports of viral infections in COVID-19.
Prof. Regina Fölster-Holst (Germany)
Paraviral exanthem is a clinically distinctive exanthem. Viral infection is suspected but there is no direct virus-related cytopathogenic effect. Paraviral exanthem merely reflecting the immune response of the host. There are several paraviral exanthema, only a few of which have been presented, such as Gianotti-Crosti syndrome, Asymmetrical periflexural exanthem, Pityriasis rosea, Pityriasis lichenoides, Acute hemorrhacig edema of infancy, Papular purpuric gloves and socks syndrome (PPGS), Eruptive psudoangiomatosis, Eruptive hypomelanosis. The diagnostic criteria include morphology, distribution, history, general health, histology, blood test, swabs. Lots of microbes (viruses and bacteria) and vaccination (hepatitis B, measles-mumps-rubella vaccine) can be associated with Gianotti-Crosti syndrome. Viral reactivation and visceral infection are associated with Drug reaction with eosinophilia and systemic symptoms (DRESS). Viral antibody titer and virus-load correlate with the severity of DRESS. At the time of making the diagnosis, antibody titers can still be negative and the virus load without abnormalities. Virus diagnostic should be performed for the following viruses: HHV-6, HHV-7, CMV, EBV. Antiviral therapy is helpful, but only in combination with systemic corticosteroids. Very interesting case reports of paraviral exanthems have been presented, such as a case of infantile DRESS (Chow ML et al, 2018), Hemorrhagic Gianotti-Crosti syndrome in a one and half month-old infant (Sarma N et al, 2013), Gianotti-Crosti syndrome like reaction secondary to molluscum contagiosum (Estébanz A, 2020), Human herpes virus -6, -7 and Ebstein-Barr virus reactivation in pityriasis rosea during COVID-19 (2021), Unilateral mediothoracic exanthem (Chuh A et al, 2016). Different clinical presentations of parvovirus B19 infection have been presented: Erythema infectiosum (only cause), PPGS (can be cause by parvovirus B19, EBV, CMV, HHV6, Coxackie B6, Hepatitis B), Petechial exanthem in “Bathing Trunk distribution caused by Parvovirus B19 infection (Huerta-Brogeras M et al). Finally, very interesting newly described paraviral exanthems have been presented: Eruptive pseudangiomatosis association with echo- and adenovirus (Acta Derm Venerology, 2017), Eruptive angiomatosis triggered by COVID 19 vaccination (Shanshai et al, 2022) and Eruptive hypomelanosis (Eruption of hypopigmented maculae in siblings, Chuh, 2016; Eruptive hypomelanosis-first case reported outside Asia, Donne M et al, 2018).
At last, but not least, I would like to present an excellent lecture in the field of psychodermatology.
