Lupus erythematosus and paediatric dermatology

Life in Geneva feels good – it’s a truly cosmopolitan city that’s not too big, and you can even enjoy a stroll by the lake – weather permitting! But no such luck today for our two roving reporters, who had to head off to Palexpo to concentrate on the sessions on Friday’s programme…

Professor Tennstedt went to the plenary session on lupus erythematosus while Professor Yordanova listened to a variety of talks focusing on paediatric dermatology, with topics of particular interest to daily practice, including infantile scleroderma, and hard-to-treat forms of atopic dermatitis. 

 

Prof. Dominique TENNSTEDT, MD, PhD

Dermatologist, Dermatology service UCL Saint Luc, Brussels, Belgium

Lupus erythematosus in daily practice

A great talk by Dan Lipsker during one of the plenary sessions!

Lupus erythematosus (LE) is traditionally subdivided into acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE) and chronic cutaneous lupus erythematosus, also known as discoid lupus erythematosus (DLE). There are also transient forms and rarer forms: lupus erythematosus tumidus (LET), lupus erythematosus panniculitis (lupus erythematosus profundus), etc.

Histologically, the interface is always affected.

Regarding ACLE, the following lab tests should be performed:

• Blood count
• Blood creatinine, GFR, urine proteins and creatinine, urine sediment
• Triglycerides, HDL, LDL, glycaemia
• C3, C4, ANA, anti DNA, anti-Ro/SSA, anti-La/SSB, aPL, beta-2-GP1

Recommended steps to be taken by patients suffering from LE:

• Protection from the sun is crucial
• Vitamin D supplements
• Stop smoking and monitor cardiovascular function
• Encourage physical activity
• Encourage anti-HPV vaccination
• Continue with oral contraception unless history of thrombosis or presence of antiphospholipid antibodies

Treatment:

• For SCLE and DLE, topical corticoids or even topical anticalcineurins can sometimes suffice.
• Antimalarial drugs are still the key treatment for SCLE and DLE.
• As a rule, the recommended dose of hydroxychloroquine is 5 mg/kg/day.
• In practice, 2 x 200 mg/day is usually prescribed (to be taken with meals). Patients must undergo an ophthalmological examination.
• If, after 4 to 6 months, there is no response, 100 to 200 mg per day of quinacrine can also be taken, if any is available!
• Chloroquine is sometimes suitable, at a dose of 2 x 100 mg/day.
• Other possibilities include: dapsone, MTX, thalidomide, lenalidomide (accompanied by small doses of AAS).
• In rare cases, certain other products might prove useful:

mycophenolate mofetil, steroids, IGIV, acitretin, alitretinoin, belimumab.

Ultraviolet-A1 irradiation therapy can also be tried.

• If there is also neutrophilic urticaria, the use of dapsone or colchicine is indicated.

Remember:

• ACLE’s main complication is the occurrence of thromboses that can result in morbidity or even mortality!
• The main signs on the skin that might suggest the potential presence or future development of thrombosis are as follows:

- pseudo atrophie blanche

- cutaneous necrosis

- filiform haemorrhages

- pseudo Degos disease

- livedo racemosa

- telangiectasia

- non infiltrated purpura

- thrombophlebitis

- ulcers

- anetodermia

In all cases, anticoagulation treatment must be given.

 

Prof. Ivelina YORDANOVA, MD, PhD

Dermatologist, Department of Dermatology and Venereology, Medical University, Pleven, Bulgaria 

Reumatology in pediatric dermatology

In the afternoon session on 15.09.2017: Paediatric dermatology - Review and Updates, Dr. Lisa Weibel - a pediatric dermatologist from University Children Hospital Zurich Switzerland presented a brilliant interractive lecture on the topic Reumatology in Pediatric Dermatology. A wide spectrum of diseases is included in differential diagnosis in skin rashes of the face, trunk and extremities in children. Among them are Lupus erythematosus, Localized screroderma and other connective tissue autoimmune disorders.

The highlight of Dr. Lisa Weibel’s lecture was the Localized scleroderma which she is very interested as a pediatric dermatologist. Localized scleroderma is clinically presented by different types: Plaque morphea, Pansclerotic morphea (Deep morphea), Linear scleroderma „en caup de sabre“ and Perry-Romberg syndrome (progressive hemifacial atrophy). Among all the cases with Localized scleroderma, 65% of them are taken by the Linear scleroderma which is usually a single, unilateral atrophic skin lesion of linear distribution involving the extremities, face or scalp. Lesions often follow Blaschko´s lines. According to the last classification of the Scleroderma, Linear scleroderma “en caup de sabre” and Perry-Romberg syndrome (progressive hemifacial athrophy) are in the spectrum of one disease but have a little difference in the characteristics. When the disorder completely affects the half of the face, it is classified as progressive hemifacial athrophy or Parry-Romberg syndrome. Is the Linear scleroderma a system disease? In order to answer this question many genetic researches of patients suffering from Linear scleroderma were conducted to establish the genetic architecture of linear localized scleroderma. Skin biopsies from 20 children were genetically investigated. The results were not deffinitive and so far no specific genetic mutation of the disease have been found. It was established that in none of the cases the disease progressed to systemic scleroderma. 20% of the patients with linear localized scleroderma in the areas of the face and scalp have neurological complication as well as ophthalmic complications in around 15%. Facial Becker’s nevus which mimics the Perry-Romberg syndrome as well as Facial partial vitiligo should be observed in the differential diagnosis. Neurological and ophthalmic complications seriously damage the quality of life of the children, their social and school adaptation which leads to holistic approach in the treatment. Treating linear localized scleroderma on the face is a challenge. Dr. Lisa Weibel explained that misdiagnosis and delay in referral of children with localized scleroderma leads to quick progression of deformations in the facial skull during the growing years. Treatment is systemic and includes combination of system corticosteroids and Methotrexate. They are applied in intravenos pulses of Methylprednisolone in dose 30 mg/kg per infusion and maximum of 500-1000 mg per day in a 3 day course continuing 3-6 months. It is followed by maintenance therapy of Methylprednisolone 0,7-1,5 mg/kg per day and the doses is gradually reduced in period of 2-4 weeks for the next 2-3 months. The pulse therapy with systemic corticosteroids is combined with Methotrexate 15 - max 20 mg/per week. It should be take subcutaneously with folic acid which reduces the side effects illnes, nausea, vomitting. The result of the 6-18 month treatment is delaining the progression of the disease. How long can the treatment with Methotrexate continue? Most commonly the treatment continues around 2-3 years and after the end of the therapy relapses are found in 25-30% of the cases. Practice shows that the cytostatic treatment can not prevent the relapse progression. In cases of resistance to Methotrexate, Mycofenolat mophetyl or Abatacept (still in clinical trial) can be used. In cases of residual deformations after the end of the growth, transplantation of patient’s own fat tissue is concider. In conclusion it is extremely important for the patient to be diagnosed as soon as possible to begin treatment - systemic cytostatic therapy of linear localized scleroderma in young age to prevent neurological and ophthalmic complications such as reducing of the facial atrophy.

Difficult to treat AD

In the morning session of Pediatric dermatology on the 15.09.2017 Dr. Kristina Sophie Ibler from Denmark presented a very useful lecture about „Treatment approaches of difficult-to-treat atopic dermatitis“. Dr. Ibler explained that most of the cases of Atopic dermatitis are represented as mild to moderate diseases, but there is a low percentage of cases when children suffer from a very severe form of Atopic dermatitis. In these cases a system therapy should be conducted in the beginning of the disease in order to improuved the quality of life of the patients, their parents and family. It is very difficult to deside to begin a system therapy in children because of the many side effects of immunomodulators. Patients who are candidates for this therapy are the ones who suffer from resistant to local treatment atopic dermatitis and who had been exposed for a long time on local corticosteroids. A few methods are used to evaluate the severety of the disease in order to start a system therapy - SCORAD which has to be >20 for moderate AD and more than 50 for severe atopic dermatitis. Scales for grading the quality of live like QoL, DLQI, Skindex 16 (impact emotions, daily living activities, social functioning) as well as Self assessment score of patients are used. According to Dr. Ibler a holistic approach should be used but also the frequency and intensity of the symptoms /itch, pain, sleep, disruptions/ have be assessed. Before starting the system treatment it is very important to teach the family and the patients themselves about the applying of adequate quantity of local basis emollient as well as preventing the patient from the so called corticophoby. A skin biopsy and conformation of the disease is mandatory before the start of the therapy. It is necessary to exclude all trigger factors - local detergents, food, stress et. Every bacterial, virus or mycotic infections should be treated before. When the disease relapses during a proactive treatment a system therapy should be induced. The choice of system medicine should be based on age, comorbidities, phase of the disease. The most commonly used systemic drugs are Cyclosporin Azathyoprin, Mycofenolate mophetyl, Methotrexate.