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A RARE DISEASE OF CONGENITAL HISTIOCYTOSIS FROM LANGERHANS CELLS IN A NEW-BORN BABY

  • Ольга Александровна Загородникова,
  • Яков Яковлевич Яковлев,
  • Елена Николаевна Гаврилова,
  • Елена Петровна Шабашкевич,
  • Ирина Валерьевна Осокина

Journal volume & issue
Vol. 23, no. 3
pp. 169 – 174

Abstract

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Histiocytosis from Langerhans cells (НCL) is a rare disease. HCL is not a hereditary, genetically determined disease. The epidemiology of HCL has not been studied enough. Purpose of the study – the aim is to present a clinical case of Langerhans cell histiocytosis (HCL) in a newborn and describe the difficulties of its diagnosis. Basic provisions. A clinical case of HCL in a newborn boy is presented. Leading in the clinic at birth was a skin syndrome in the form of hemorrhagic rash and polymorphic, common elements on the skin of the trunk, limbs, scalp, face. Initially, a diagnosis of herpes infection was made. However, due to the lack of positive results of PCR diagnostics, as well as doubts about the reliability of the diagnosis, the infant was discharged with a formula for the final diagnosis of infectious vasculitis of unknown etiology. Later, at the age of two months, when the patient had complications with multisystem damage to the bones of the skeleton, lungs, bone marrow, according to the results of histological and immunohistochemical studies, HCL was diagnosed. Conclusion. HCL is a rather rare pathology, the clinical picture of which is diverse and nonspecific, which causes difficulties in diagnosis. In the presented case, a biopsy of the skin elements should have been performed for early diagnosis, which would have allowed to make the correct diagnosis and prevent complications.

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