Download fulltext HTML
H H, S., Elamin, M. M., Modawe, G. A., & Elseed, K. A. A. (2018). Letterer Siwe Disease (LSD): A Case Report. Sudan Journal of Medical Sciences (SJMS), 13(3), 207–218. https://doi.org/10.18502/sjms.v13i3.2958

https://doi.org/10.18502/sjms.v13i3.2958

statistics

  • 1558 Abstract Views
  • 442 PDF Views
  • 0 HTML Views

authors

  • Suad H H
    No author data available.
  • Mona Mohamed Elamin
    No author data available.
  • Gad Allah Modawe
    No author data available.
  • Khalid AbdElmohsin Awad Elseed
    No author data available.

Published Date

  • Sep 24, 2018

Letterer Siwe Disease (LSD): A Case Report

Sudan Journal of Medical Sciences (SJMS) / Sudan JMS: Volume 13 (2018), Issue No. 3 / Pages 207–218

Abstract

Background: Letterer–Siwe Disease (LSD) is one of the variants of Langerhans cell histiocytosis (LCH), which is considered as a rare disease that affects many systems in the body; it is characterized by monoclonal migration and proliferation of specific dendritic cells. The disease affects the bones and skin primarily, but can involve
other organs as well, or appear as a multi-system disease leading to different clinical manifestations and eventually death.


Summary: The authors present a case report of LSD in a two-year-old child from western Sudan, Messeria tribe, who is presented with one and a half-month history of fever, cutaneous ulcers, purprae,
scaly crusted scalp, and pallor. His full blood count showed very low Hb with marked reduction of platelets. TWBC was normal. US showed hepatosplenomegaly with lymphadenopathy. A suspicion of sickle cell anemia and leukemia was suggested. He received treatment in his area in the form of antibiotics, skin care, blood transfusion and platelets aggregate without improvement. Patient was referred to Khartoum for further investigations and management. On presentation, a diagnosis of histiocytosis x was suggested depending on the clinical presentation of a general ill health in a child with purpurae, skin ulcers, and a scaly crusted scalp. A skin biopsy, bone marrow aspirate, and a skull x-ray were requested. Bone marrow aspiration showed hyper cellular BM with marked hemophagocytosis. Patient was admitted in a pediatric ward for further general investigations and blood transfusion, but he passed few days later before starting chemotherapy. Usually this is the prognosis of this rare and fatal aggressive form of histiocytosis x.


Conclusion: A sick child with fever, anemia, hepatosplenomegaly, scaly scalp, and skin lesions should be investigated for LSD.

References
[1] Shooriabi, M., Parsazade, M., Bagheri, S., et al. (2016). Langerhans cell histiocytosis in childhood: Review, symptoms in the oral cavity, differential diagnosis and report of one case. International Journal of Pediatrics, vol. 4, no. 8, pp. 3343–3353.

[2] Shahlaee, A. H. and Arceci, R. J. (2007). Histiocytic disorders, in Pediatric Hematology (third edition), p. 340359. John Wiley and Sons.

[3] Laman, J. D., Leenen, P. J., Annels, N. E., et al. (2003). Langerhans-cell histiocytosis ’insight into DC biology’. Trends in Immunology, vol. 24, no. 4, p. 190.

[4] Geissmann, F., Lepelletier, Y., Fraitag, S., et al. (2001). Differentiation of Langerhans cells in Langerhans cell histiocytosis. Blood, vol. 97, no. 5, pp. 1241–1248.

[5] Berres, M. L., Allen, C. E., and Merad, M. (2013). Pathological consequence of misguided dendritic cell differentiation in histiocytic diseases. Advances in Immunology, vol. 120, pp. 127–161.

[6] Willman, C. L., Busque, L., Griffith, B. B., et al. (1994). Langerhans’-cell histiocytosis (histiocytosis X)-a clonal proliferative disease. The New England Journal of Medicine, vol. 331, no. 3, pp. 154–160.

[7] Yu, R. C., Chu, C., and Buluwela, L. (1994). Clonal proliferation of Langerhans cells in Langerhans cell histiocytosis. Lancet, vol. 343, no. 8900, pp. 767–768.

[8] PDQ® Pediatric Treatment Editorial Board. PDQ Langerhans Cell Histiocytosis Treatment. Bethesda, MD: National Cancer Institute. Retrieved from http://www. cancer.gov/types/langerhans/hp/langerhans-treatment-pdq (accessed on April 2013 [Updated on June 9, 2016]).

[9] Hammar S. P. and Allen T. C. (2008). Histiocytosis and storage diseases, in J. F. Tomashefski, P. T. Cagle, C. F. Farver, A. E. Fraire (eds.) Dail and Hammar’s Pulmonary Pathology. New York, NY: Springer Science Business Media, LLC.

[10] Olschewski, T., Micke, O., Seegenschmiedt, M. H. (2008). Langerhans’ cell histiocytosis, in M. S. Seegenschmiedt, H.-B. Makoski, K.-R. Trott, and L. W. Brady (eds.) Radiotherapy for Non-Malignant Disorders. New York, NY: Springer Berlin Heidelberg.

[11] Abt, A. F. and Denenholz, E. J. (1936). The American Journal of Diseases of Children, vol. 51, p. 499; Curtis, A. C. and Cawley, E. P. (1947). Arch. Derm. Syph. (Wien), vol. 55, p. 810; Farber, S. (1941). American Journal of Pathology, vol. 17, p. 625; Flori, A. G. and Parenti, G. C. (1937). Rivista di Clinica Pediatrica, vol. 35, p. 193. (Cited by Jaffe and Lichtenstein.)

[12] Pant, C., Madonia, P., Bahna, S. L., et al. (2009). Langerhans cell histiocytosis, a case of Letterer Siwe disease. Journal of the Louisiana State Medical Society, vol. 161, no. 4, pp. 211–212.

[13] Reserved, Inserm US14- All Rights. Orphanet: Letterer Siwe Disease. Retrieved from www.orphanet (accessed on May 19, 2017).

[14] Ferreira, L. M., Emeriti, P. S., Diniz, L. M., et al. ( July/August 2009). Anais Brasileiros de Dermatologia, vol. 84, no. 4. Rio de Janeiro

[15] Plotski, A., Lupachick, E., Vasilykononov, et al. (2013). Archives of Perinatal Medicine, vol. 19, no. 1, pp. 55–57.