C007 Atopic eczema in Russia

S60

Courses

-European

Society

of Paediatric

come an essential component of our diagnostic armamentarium enabling us to readily differentiate between lamina lucida and sub-lamina densa immuno-deposits. Electron microscopy, immuno-electron microscopy and molecular biological techniques have additionally provided a greater insight into the pathogenesis of these blistering lesions. In this presentation emphasis will be placed on the clinicopathological and split-skin immunofluorescent findings in a variety of diseases including bullous pemphigoid, dermatitis herpetiformis, inflammatory variant of epidermolysis bullosa acquisita, linear IgA disease and bullous systemic lupus erythematosus.

European Society of Paediatric Dermatology: Paediatric Dermatology Childhood IICOO5 I

mastocytosis

Arnold P. Oranje. Department (Paediatric Dermatology and Erasmus University,

of Dermatology and Venereology Unit), University Hospital Rotterdam The Netherlands

Mastocytosis is a heterogeneous group of diseases that are characterized by mast cell proliferation in the skin and eventually other organs. The disease may start at any age, but is more common in young children. In 75% of the cases it starts below the age of two years. Histamine is an indicator for mast cell activation. N-methyl-histamine (NMH) is a metabolite of histamine, that is measured in urine. Urinary NMH values correlate with the extent and activity of the disease. In the normal regressive course of childhood mastocytosis NMH values decrease with age. High values indicate systemic involvement.

n

COO6 Langerhans cell histiocytosis in children

F. Cambazard. Service Hopital

Nord,

42055

de Dermatologie St Etienne Cedex

CHU de St-Etienne, 2, France

Langerhans cell histiocytosis (LCH) includes a wide spectrum of clinical disease: Letterer Siwe, Hand Schtiller Christian, eosinophilic granuloma of bone, and neonatal self healing histiocytosis, Lesions may be isolated or evolve to a widespread systemic disease. Every organ may be involved: skin, bone, bone marrow, liver, spleen, lung, gastrointestinal tract, central nervous system, lymph nodes. Pronostic factors, are age of onset, number of organs involved, and organ dysfunction with a mortality rate reaching 50% below 2 years of age, with four organs involved and anemia, trombenia, liver or lung dysfunction. In all cases there is a uniform pathological feature which cannot predict the clinical outcome. Langerhans cells (LC) proliferation induces an inflammatory granuloma which destroys tissues cells, leading to scar fibrotic tissue. Long-term sequellac from this destructive cicatricial process are endocrine dysfunction (diabetes insipidus, growth disorders), orthopedic abnormalities, liver fibrosis, pulmonary abnormalities, CNS disorders, vision or hearing deficits. Proliferating cells appear like normal LC (highly differentiated) with absence of heteroploid cells and metastatic dissemination. LCH seems to be more a reactive disorder (excessive immune response) than a neoplastic process even if clonality was found by molecular

Dermatology:

Paediatric

Dermatology

analysis of patterns of chromosome X in activation. Etiopathogenesis is always hypothetical and a viral pathogenesis was never proved. Therapy is surgery or radiation (isolated lesions), sytemic chemotherapy or topical nitrogen mustard which gives a rapid clinical skin improvement. Chemotherapy are more aggressive if there is an organ dysfunction but may induce introgenic complications such as acute myeloid leukemia after etoposide. Malignant neoplasm as solid tumor, lymphema or leukemia may alter the prognosis. New therapy as ciclosporine or medullary transplantation may be effective ElCOO7 Atopic eczema in Russia N.P. Toropova, O.A. Sinyavskaya, A.M. Gradinarov’, D.I. Zelinskaya*. ‘Medical Centre, Ekaterinburg; *Ministry Health,

Moscow,

of

Russia

Prevalence of atopic eczema (AE) varies between 11.2% and 27.0% in different age groups. The difference in AE incidence throughout the country is due to environmental pollution, nutrition, endemic infections, climate, social and economic situation etc. Because of the great significance of the problem, AE research is supported by the state. There are specialised centres and several scientific schools dealing with AE. In recent years, a significant pathomorphosis in the clinical course of AE has been noted: new variants of AE have been registered, the number of patients with severe AE has considerably increased. Our concept of AE pathogenesis takes into account hereditary predisposition, perinatal disorders, immune system indices etc. Special attention is paid to visceral pathology as the source of endogenic autoallergens and biologically active substances, and neurovertebral dysfunctions resulting in microcirculatory and trophic skin disorders. The main principle of AE therapy is recovery of the skin, viscera, immune and nervous system functioning. Preference in topical therapy is given to non-steroid agents. ElCOO8 Genetic disorders predisposing

to skin

malignancies

John Harper. England Abstract not available. ElCO09 Paediatric HIV infection K.M. Butler. Our Lady’s Hospitalfor Sick Children Children’s

Hospital,

Dublin,

and the

Ireland

In the 20 years since the first cases of AIDS were reported the WHO estimates that over 20 million adults and 1.5 million children have been infected and more than one thousand children a day are born with HIV. The incidence is highest in sub-Saharan Africa and south Asia and continues to rise in India. In the developed world there has been a leveling off in the number of new cases in gay/bisexual men. The proportion of new cases in women continues to rise alarmingly. As virtually all cases in children are the result of vertical transmission, this growth in numbers of infected women represents a serious threat to child-health. Vertical transmission rates, influenced by