Ahmed Yasser Nada
RUDN University, Russian FederationPresentation Title:
Diffuse cutaneous mastocytosis in children: dynamics of tryptase levels and frequency of anaphylaxis
Abstract
Relevance-Diffuse cutaneous mastocytosis (DCM) is a rare clinical form of mastocytosis (0.5% of all cases of the disease). High risk of severe systemic mediator-related symptoms and anaphylaxis requires careful monitoring of children.
Material and methods.- We observed 5 children (4 boys and 1 girl) aged from 2 months to 7 years at the Moscow Scientific and Practical Center of Dermatovenereology and Cosmetology. The diagnosis in all children was confirmed by pathomorphological examination. Immunohistochemical staining with antibodies to CD117 revealed a positive reaction in the vast majority of dermal infiltrate cells. Serum tryptase level was determined by immunofluorescence on a three-dimensional porous solid phase.
Results-In all children, mastocytosis debuted at birth. In infancy and early childhood, all children had predominantly extensive bullous lesions. Bullous lesions persisted in children up to 5 years of age. In children over 1 year of age, diffuse cutaneous infiltration with lichenification and hyperpigmentation of varying severity was predominant. At the first visit, the average tryptase level was 19.3 ng/l. During dynamic observation, a gradual increase in tryptase levels was noted in two patients during the first three years, while a decrease was observed in the rest. Multiple episodes of anaphylaxis were recorded in two children. Other systemic mediator-related symptoms (abdominal pain, diarrhea, impaired concentration and sleep, prolonged flushing, syncope) of varying severity were observed in all patients. Ultrasound signs of hepatomegaly and splenomegaly were detected in 4 children.
Conclusions-Management of patients with DCM should include dynamic observation by a pediatric dermatologist and allergist-immunologist, monitoring of the functions of internal organs and peripheral blood indices, dynamic monitoring of tryptase levels to exclude systemic mastocytosis and the prescription of anti-mediator-related and external therapy corresponding to the nature of the cutaneous pathological process.
Biography
Ahmed Yasser Nada in process of PHD at the age of 26 years from RUDN University, Russia. I am in 2nd course of PHD at department of Dermatovenerology and cosmetology at RUDN University, Russia. I have 2 publications that have been cited over 2 times and I presented many presentations at different conferences of Dermatovenerology and Cosmetology around the World.