Letterer-Siwe disease presenting with gastrointestinal and cutaneous manifestations

Histiocytosis is a set of distinct proliferative illnesses defined by the proliferation and infiltration of varied numbers of dendritic cells, macrophages, and monocytes in the afflicted tissues. The skin and other organs may be impacted by the inflammatory infiltration. It can occur at any age. The severity of the symptoms can range from mild to severe, depending on the degree and type of organ involvement. Although certain forms of histiocytosis can be fatal, others can be treated successfully without sequelae. Langerhans cell histiocytosis manifests itself clinically in both children and adults. A combination of clinical, histological, and radiological tests is required to achieve a diagnosis. A severe, multisystemic, acute form of Langerhans cell histiocytosis is called Letterer-Siewe illness, which usually affects infants in their first year of life. In this article, we provide a brief literature review and a case study of a 9-month-old girl who presented with recurring gastrointestinal problems as the first sign of Letterer-Siwe disease. © 2023 Dermatology Online Journal. All rights reserved.

Authors
Stadnikova A.S. , Abbas W.F. , Tamrazova O.B. , Pristanskova E.A. , Zakharova I.N. , Berezhnaya I.V. , Skobeev D.A. , Goncharova L.V.
Publisher
Dermatology Online Journal
Number of issue
6
Language
English
Status
Published
Volume
29
Year
2023
Organizations
  • 1 Federal State Autonomous Educational, Institution of Higher Education, "Peoples' Friendship University of Russia", the Ministry of Education and Science of the Russian Federation, Children's City Clinical Hospital named after PER, Bashlyaeva, Moscow Department of Health, Moscow, Russian Federation
  • 2 Department of Dermatovenerology with Course Cosmetology, RUDN university, Moscow, Russian Federation
  • 3 Russian Children's Clinical Hospital, the Federal State Autonomous Educational, Institution of Higher Education, Russian National Research Medical University named after NI Pirogov", the Ministry of Health of the Russian Federation, Moscow, Russian Federation
  • 4 Department of Pediatrics named after Academician, GN Speransky FGBOU DPO RMANPO, the Ministry of Health of Russia, Moscow, Russian Federation
  • 5 Pathoanatomical Department, the Morozov Children's Clinical Hospital, Moscow, Russian Federation
Keywords
gastrointestinal; histiocytosis; Langerhans cell; Letterer-Siwe disease; seborrheic dermatitis; skin rash
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